Geriatric Otolaryngology

Geriatric Otolaryngology

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Geriatric Otolaryngology edited by

Karen H. Calhoun, M.D. University of Missouri Columbia, Missouri, U.S.A.

David E. Eibling, M.D. University of Pittsburgh Pittsburgh, Pennsylvania, U.S.A. Associate Editors

Mark K. Wax, M.D. Oregon Health and Science University Portland, Oregon, U.S.A.

Karen M. Kost, M.D. McGill University Montreal, Quebec, Canada

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Published in 2006 by Taylor & Francis Group 270 Madison Avenue New York, NY 10016 © 2006 by Taylor & Francis Group, LLC No claim to original U.S. Government works Printed in the United States of America on acid-free paper 10 9 8 7 6 5 4 3 2 1 International Standard Book Number-10: 0-8247-2850-5 (Hardcover) International Standard Book Number-13: 978-0-8247-2850-2 (Hardcover) This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted with permission, and sources are indicated. A wide variety of references are listed. Reasonable efforts have been made to publish reliable data and information, but the author and the publisher cannot assume responsibility for the validity of all materials or for the consequences of their use. No part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, please access www.copyright.com (http://www.copyright.com/) or contact the Copyright Clearance Center, Inc. (CCC) 222 Rosewood Drive, Danvers, MA 01923, 978-750-8400. CCC is a not-for-profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Library of Congress Cataloging-in-Publication Data Catalog record is available from the Library of Congress

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To all senior otolaryngologists who, like Sid Busis, continue to practice into their 70s and 80s.

Preface

Sidney Busis The goal of health care is to prolong life and relieve suffering. Each of us hopes to someday achieve elderly status, since the alternative is a life shortened by illness or other misfortune. Yet, simply achieving an advanced age is an imperfect goal for both our patients and ourselves. Our real aim, and that of our patients as well, is to live a rewarding and useful life for as long as is possible. This text is a guide for practitioners in our specialty to assist them in helping their elderly patients achieve their goal of health and the associated high levels of function for as long as possible. By doing so, our patients, and hopefully we as well, will continue to enjoy life and contribute in a meaningful manner to their (and our) professions, families, and communities. Each of us personally knows individuals who are role models in this regard and have been able to contribute much even in their advanced years. This theme of long-term contribution to a community, even into advanced age, is perhaps demonstrated no better than by Sidney Busis, the author of Chapter 6 ‘‘Presbycusis’’ in this book. ‘‘Sid’’ was a solo private practitioner of otolaryngology for 54 years in Pittsburgh prior to his retirement at the end of 2004. At the age of 84 he continues to be an active contributor to his community, and to this book. Sid Busis was born September 2, 1921, grew up in Pittsburgh, and attended the University of Pittsburgh for both undergraduate and medical school. During medical school he was an active-duty private in the army, and upon graduation in 1945 vii

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he was promoted to first lieutenant and assigned to Randolph Air Force Base where he trained to be a flight surgeon. In 1948, after discharge from the Army Air Corps, he began his training in otolaryngology at the University of Pittsburgh. During his residency he also found time for additional specialty training at the University of Pennsylvania and spent a summer with Chevalier Jackson, who was at Temple University. Following his residency Sid entered practice and became a solo practitioner of general otolaryngology, later focusing his practice on diseases of the ear. He was a busy surgeon, often performing more than 700 procedures yearly. Although he was in private practice, he actively taught residents both in the operating room and in his office, directed many courses in Pittsburgh, and gave invited lectures. He contributed to the otolaryngology literature and presented instruction courses at the annual meeting of the American Academy of Otolaryngology yearly for 29 years. He also served as president of the medical staff of the Eye and Ear Hospital of Pittsburgh and as president of the American Neurotology Society. In 1972 Sid recruited Eugene Myers to the University of Pittsburgh as chairman and the first full-time faculty member in the department. Over the next 33 years Dr. Myers served as department chair, and he and Sid remain close friends to this day. Sid did much more for his community than practice otolaryngology and teach. He was, and remains, active in his local temple and community, serving as president of the United Jewish Federation of Pittsburgh and president of his congregation. He has donated both time and money through his leadership of local fund raising campaigns as well as several national nonprofit organizations. Sid has been a lifelong optimist. When he says ‘‘Do the math’’ he is not referring to the funds he has collected or to his years in practice, but rather to how lucky we all are to be alive at this time. ‘‘Do the math’’ means ‘‘Count your blessings,’’ a message for all of us, regardless of age. The significance of Sid’s long career cannot be underestimated. Not only did he contribute in a meaningful way to his patients, his residents, and his community, but he continued to do so until into his eighties. His professional activities have spanned nearly 60 years, 54 years in solo private practice. When asked to author a chapter for this book when already 20 years past the age when most individuals are considering retirement, he eagerly accepted and in a short time produced a scholarly work. As you read his chapter, recall that Sid finished medical school at the end of World War II and that he has been in practice since before many or most of us were born. The lifelong continuing contributions of Sidney Busis have served as an inspiration to the editors of this text. We hope that we, too, will be able to contribute over the years as he has shown to be possible. As you read Sid’s chapter, be reminded that many of our geriatric patients are capable and willing to contribute to their professions, families, and communities. Assisting them in achieving this goal through appropriate management of diseases and disorders of the ear, nose, and throat is, in fact, our responsibility as practitioners of our specialty. Sid reminds us of what is possible for our patients, and ourselves, if we only believe it to be so. Karen H. Calhoun David E. Eibling Karen M. Kost Mark K. Wax

M.D. M.D. M.D. M.D.

Contents

Preface . . . . vii Contributors . . . . xxix PART I: GERIATRIC OTOLARYNGOLOGY 1. Clinical Approach to the Geriatric Patient . . . . . . . . . . . . . . . . . . . 1 Michelina Fato Demographics of Aging in America . . . . 2 Practical Approach to the Office Visit . . . . 4 Obtaining the History . . . . 5 Social History . . . . 6 Nutrition . . . . 6 Alcohol, Tobacco, and Drug Use Disorders . . . . 7 Functional Status . . . . 7 End of Life . . . . 7 Physical Examination . . . . 8 Elder Abuse and Neglect . . . . 8 Depression and Suicide . . . . 9 Summary . . . . 9 Appendix . . . . 10 References . . . . 14 2. Biology of Aging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 Mukaila A. Raji and James S. Goodwin Introduction . . . . 17 Life Expectancy, Life Quality, and Life Span . . . . 17 Theories of Aging . . . . 18 Antiaging Interventions—Insight and Potentials from Aging Theories . . . . 22 Summary and Conclusion . . . . 23 References . . . . 23 3. Introduction to Geriatric Otolaryngology . . . . . . . . . . . . . . . . . . . 25 Hinrich Staecker Introduction . . . . 25 ix

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The Auditory System . . . . 25 Vestibular System . . . . 31 The Nose and Sinuses . . . . 32 Tongue/Oral Cavity and Swallowing . . . . 33 Larynx . . . . 35 Conclusions . . . . 37 References . . . . 37 4. Cerumen Impaction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 Cyrus Torchinsky and Terence M. Davidson Introduction . . . . 43 Anatomy and Physiology of Cerumen . . . . 43 Pathophysiology . . . . 47 Incidence . . . . 48 Treatment . . . . 49 Complications of Cerumen Removal . . . . 50 Our Practice Algorithm . . . . 51 Historical Precedent and Conclusions . . . . 52 References . . . . 56 5. Amplification and the Geriatric Patient . . . . . . . . . . . . . . . . . . . . 59 Catherine V. Palmer and Amanda Ortmann General Description of the Clinical Problem . . . . 59 Why Is Hearing Loss Particularly Important in the Geriatric Population? . . . . 59 Basic Science . . . . 60 History . . . . 60 Physical Examination . . . . 62 Testing . . . . 62 Treatment Options . . . . 63 References . . . . 73 Annotated Bibliography . . . . 74 6. Presbycusis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 Sidney N. Busis Incidence . . . . 77 Recognition . . . . 78 Peripheral Pathology . . . . 79 Audiometric Patterns . . . . 80 Central Auditory Pathways . . . . 80 Molecular Mechanisms . . . . 82 Genetic Factors . . . . 83 Evaluation . . . . 83 Management . . . . 85 References . . . . 88

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7. Tinnitus in the Elderly Patient . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Steven M. Parnes Tinnitus . . . . 91 Tinnitus That Originates from Para-auditory Structures (Objective Tinnitus) . . . . 92 Sensorineural (Subjective) Tinnitus . . . . 94 Conclusions . . . . 98 References . . . . 98 8. Hearing Protection for the Retired Adult . . . . . . . . . . . . . . . . . . 101 Marshall Chasin and Joanne DeLuzio Introduction . . . . 101 Hearing Protection . . . . 102 Acoustically (and Electronically) Tuned Hearing Protection . . . . 104 Rating Hearing Protection . . . . 106 Strategies for Grandchildren to Protect Their Hearing . . . . 107 References . . . . 108 9. Multisensory Impairment in Older Adults: Evaluation and Intervention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109 Susan L. Whitney and Laura O. Morris Introduction . . . . 109 Normal Age-Related Changes . . . . 110 Pathological Sensory Changes in the Older Adult . . . . 111 Somatosensory Screening . . . . 111 Visual Screening . . . . 113 Vestibular Screening for Older People . . . . 115 Intervention for Multisensory Disequilibrium . . . . 118 References . . . . 121 Annotated Bibliography . . . . 122 10. Mal de Debarquement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125 Timothy C. Hain and Dario A. Yacovino General Description of the Clinical Problem . . . . 125 Basic Science . . . . 125 Potential Etiologic Factors in MDD . . . . 127 Diagnosis of MDD . . . . 130 Treatment of MDD . . . . 131 Algorithm . . . . 132 Summary . . . . 133 References . . . . 133 11. Meniere’s Disease in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . 135 Ronen Perez and Julian M. Nedzelski Epidemiology . . . . 135 Pathogenesis . . . . 136 Diagnosis . . . . 137

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Treatment . . . . 140 Summary . . . . 142 References . . . . 143 Annotated Bibliography . . . . 145 12. Dizziness in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 David E. Eibling Introduction . . . . 147 Taxonomy of Dizziness in the Elderly . . . . 149 Role of the Otolaryngologist in Non-vestibular Dizziness . . . . 150 Approach to the Patient with Non-vestibular Dizziness . . . . 151 Treatment of Non-vestibular Dizziness . . . . 152 Summary . . . . 153 References . . . . 153 13. Benign Paroxysmal Positional Vertigo . . . . . . . . . . . . . . . . . . . . 155 Joseph M. Furman General Description of the Clinical Problem . . . . 155 Pathophysiology of BPPV . . . . 156 Clinical Presentation . . . . 157 Treatment of BPPV . . . . 159 References . . . . 162 Annotated Bibliography . . . . 164 14. Balance Rehabilitation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165 Horst R. Konrad and Meiho Nakayama Introduction . . . . 166 General Description of the Clinical Problem . . . . 167 Summary . . . . 170 References . . . . 171 PART II: TASTE, SMELL, AND NASAL PROBLEMS 15. Olfaction and Aging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173 Karen J. Fong and Mark A. Zacharek Introduction . . . . 173 Definitions . . . . 174 Etiologies of Olfactory Loss . . . . 174 Pathophysiology of Olfactory Loss with Aging . . . . 174 Patient Evaluation . . . . 176 Treatment and Counseling . . . . 177 References . . . . 179 16. Olfactory Dysfunction in Neurodegenerative Diseases Richard L. Doty Introduction . . . . 181

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Neurodegenerative Diseases with Marked Olfactory Dysfunction . . . . 182 Neurodegenerative Diseases with Less Marked or Minimal Olfactory Dysfunction . . . . 187 Conclusions . . . . 190 References . . . . 191 17. Taste Changes in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . . 195 Natasha Mirza Introduction . . . . 195 Development of the Taste System . . . . 195 Anatomy of the Taste System . . . . 196 Taste Transduction . . . . 197 Innervation of the Tongue . . . . 198 Review of Literature . . . . 199 Reasons for Loss of Taste in the Elderly . . . . 200 Effects of Taste Loss in the Elderly . . . . 201 Therapy for Taste Loss . . . . 202 Conclusion . . . . 202 References . . . . 202 18. Sinusitis: Diagnosis, Treatment, and Complications . . . . . . . . . . . 205 Berrylin J. Ferguson and Umamaheswar Duvvuri Introduction . . . . 205 Acute Sinusitis . . . . 205 Antibiotic Selection . . . . 207 Chronic Sinusitis . . . . 209 Chronic Fungal Rhinosinusitis . . . . 210 Chronic Invasive Fungal Sinusitis . . . . 211 Summary . . . . 212 References . . . . 212 19. Allergic Rhinitis in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . 213 Stephen J. Chadwick Background . . . . 213 Patient Presentation and History . . . . 215 Diagnosis . . . . 217 Management . . . . 217 Immunotherapy . . . . 219 Food Allergy . . . . 220 Summary . . . . 220 References . . . . 221 20. Rhinitis in the Aging Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . 225 Margaret A. Chen and Terence M. Davidson Introduction . . . . 225

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Overview of Normal Nasal Physiology . . . . 225 Pathophysiology of Aging Rhinitis . . . . 226 Clinical Assessment . . . . 227 Management . . . . 228 Conclusion . . . . 235 References . . . . 236 21. Epistaxis in the Geriatric Population . . . . . . . . . . . . . . . . . . . . . 239 Dana S. Smith and Mark K. Wax Introduction . . . . 239 Anatomy . . . . 239 Etiology . . . . 240 Evaluation of Epistaxis . . . . 241 Treatment of Epistaxis . . . . 242 Summary . . . . 245 References . . . . 246 PART III: ORAL DISORDERS 22. Oral Mucosal Pathology in the Geriatric Population . . . . . . . . . . 249 Bobby M. Collins Herpes Simplex . . . . 250 Erythema Multiforme . . . . 251 Pemphigoid . . . . 252 Pemphigus . . . . 252 Lichen Planus . . . . 254 Lichenoid Drug Reaction . . . . 256 Aphthous Ulcers . . . . 256 Biopsy . . . . 257 Selected Suggested Medications . . . . 258 Bibliography . . . . 259 23. Halitosis in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261 David E. Eibling Introduction . . . . 261 Approach to the Patient with Halitosis . . . . 262 Treatment of Halitosis . . . . 262 Conclusion . . . . 264 References . . . . 264 24. Oral Health Care of the Elderly Patient . . . . . . . . . . . . . . . . . . 265 Azfar A. Siddiqui Introduction . . . . 265 Dental Loss in the Elderly . . . . 265 Changes in Oral Dental Health with Aging . . . . 265 Dentures in the Elderly . . . . 266

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Treatment of Denture Problems in the Elderly . . . . 266 Dental Caries . . . . 267 Treatment . . . . 268 Periodontal Disease . . . . 268 Treatment of Periodontal Disease . . . . 268 Oral Health of Cancer Patients . . . . 269 Denture Care . . . . 270 Summary . . . . 271 Reference . . . . 271 25. Burning Mouth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273 David E. Eibling Introduction . . . . 273 Representative Case . . . . 273 Epidemiology of BMS . . . . 274 Etiology . . . . 274 Evaluation and Management of Patients with BMS . . . . 275 Summary . . . . 276 References . . . . 276 PART IV: VOICE AND SWALLOWING 26. Effects of Aging on Swallowing . . . . . . . . . . . . . . . . . . . . . . . . . 277 JoAnne Robbins, Nadine Connor, and Steven Barczi Introduction . . . . 277 The Impact of Dysphagia . . . . 278 Normal Swallowing . . . . 278 Neurophysiology of Oropharyngeal Swallow . . . . 280 Senescent Swallowing . . . . 281 Differential Considerations for Dysphagia and Aspiration . . . . 285 Dysphagia Intervention–Prevention Paradigms . . . . 287 Summary . . . . 288 References . . . . 288 27. Dysphagia Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 Jonathan E. Aviv and Thomas Murry Introduction . . . . 293 Epidemiology . . . . 293 Etiology . . . . 294 Diagnostic Techniques . . . . 295 Treatment . . . . 296 Summary . . . . 299 References . . . . 299 28. Xerostomia in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Karen M. Kost Introduction . . . . 303

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Physiology . . . . 303 Etiology . . . . 304 Clinical Consequences of Xerostomia . . . . 307 The Work Up of Xerostomia . . . . 307 Management of Xerostomia . . . . 308 Oral Care—Topical Agents . . . . 309 Oral Care—Systemic Agents . . . . 309 Other Treatments . . . . 310 Summary . . . . 310 References . . . . 311 29. Zenker’s Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Daniel H. Coelho, Douglas A. Ross, and Clarence T. Sasaki General Description of the Clinical Problem . . . . 313 Why Is Zenker’s Diverticulum Particularly Important in the Geriatric Population? . . . . 313 Basic Science . . . . 313 History . . . . 315 Physical Examination . . . . 315 Testing . . . . 316 Differential Diagnosis . . . . 316 Treatment Options . . . . 317 Algorithm . . . . 320 Conclusion . . . . 321 References . . . . 321 Bibliography . . . . 323 30. Surgical Management of Aspiration in the Geriatric Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325 David E. Eibling Introduction . . . . 325 Ethical Dilemmas . . . . 325 Approach to the Patient . . . . 326 Treatment of Intractable Aspiration . . . . 326 Vocal Cord Medialization . . . . 327 Cricopharyngeal Myotomy or Chemodenervation . . . . 327 Aerodigestive Separation . . . . 327 Laryngeal Stenting . . . . 327 Total Laryngectomy . . . . 328 Laryngeal Closure . . . . 328 Laryngotracheal Separation . . . . 328 Summary . . . . 329 References . . . . 329 31. Sialorrhea in the Geriatric Population . . . . . . . . . . . . . . . . . . . . 331 Sam J. Daniel General Description of the Clinical Problem . . . . 331

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Why Is Sialorrhea Particularly Important in the Geriatric Population? . . . . 331 Basic Science . . . . 331 History . . . . 332 Physical Examination . . . . 332 Differential Diagnosis . . . . 332 Testing . . . . 333 Treatment Options . . . . 333 Summary . . . . 335 Bibliography . . . . 335 32. Post-Stroke Swallowing Rehabilitation . . . . . . . . . . . . . . . . . . . . 337 Stephanie K. Daniels and Anne L. Foundas Introduction . . . . 337 Lesion Localization and Swallowing . . . . 337 Screening for Dysphagia in Acute Stroke . . . . 339 Instrumental Evaluation of Oropharyngeal Dysphagia . . . . 341 Treatment of Oropharyngeal Dysphagia . . . . 342 Summary . . . . 344 References . . . . 345 33. The Effects of Age on the Voice . . . . . . . . . . . . . . . . . . . . . . . . 349 Robert Thayer Sataloff and Sue Ellen Linville Introduction . . . . 349 Anatomy and Physiology . . . . 349 Correlates of Anatomic Changes from Young Adulthood to Old Age . . . . 352 Variability in Measures of Voice and Speech . . . . 353 The Adult Voice . . . . 353 The Aging Voice . . . . 353 Aging . . . . 354 Medical Intervention . . . . 354 Psychology and Intellect . . . . 355 Endocrine System . . . . 355 Neurologic Diseases . . . . 356 Head and Neck . . . . 356 Other Conditions . . . . 356 Voice Therapy . . . . 356 Surgery . . . . 357 Conclusion . . . . 360 References . . . . 360 34. Vocal Fold Paralysis: Surgical Treatment . . . . . . . . . . . . . . . . . . 365 Steven M. Zeitels General Description of the Clinical Problem . . . . 365 Why Is UVP Particularly Important in the Geriatric Population, Including Recent Concepts in Pathophysiology? . . . . 365

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History . . . . 366 Physical Examination . . . . 367 Differential Diagnosis . . . . 367 Testing . . . . 367 Treatment Options . . . . 368 Injection Medialization . . . . 368 Laryngoplastic Phonosurgery: Background . . . . 369 Principles and Theory of Laryngeal Framework Surgery . . . . 369 Medialization Laryngoplasty . . . . 369 Arytenoid Procedures: Posterior Glottal Incompetence . . . . 370 Cricothyroid Subluxation . . . . 371 References . . . . 372 Recommended Reading . . . . 373 35. Cough . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375 David E. Eibling Introduction . . . . 375 Cough as a Physiologic Function . . . . 375 Cough as a Symptom . . . . 376 Chronic Cough as a Complaint . . . . 376 Chronic Cough in the Elderly . . . . 378 Approach to the Patient with Cough . . . . 378 History of the Patient with Cough . . . . 378 Physical Examination of the Patient with Cough . . . . 379 Additional Testing . . . . 380 Treatment of Chronic Cough . . . . 381 Summary . . . . 381 References . . . . 382 36. Laryngopharyngeal Reflux in the Elderly . . . . . . . . . . . . . . . . . . 383 Karen M. Kost and Richard J. Payne Introduction . . . . 383 Laryngopharyngeal Reflux . . . . 384 Clinical Evaluation . . . . 386 Management of LPR . . . . 389 Summary . . . . 391 References . . . . 392 PART V: SLEEP DISORDERS 37. Identifying and Treating Sleep Disorders in the Elderly . . . . . . . . 395 Lianqi Liu, Sonia Ancoli-Israel, and Lavinia Fiorentino General Description of the Clinical Problem . . . . 395 Normal Sleep . . . . 395 Why Is Poor Sleep Particularly Important in the Geriatric Population? . . . . 396 History . . . . 396

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Differential Diagnosis . . . . 397 Testing . . . . 402 Treatment . . . . 403 Conclusion . . . . 407 References . . . . 408 38. Methodologies in the Diagnosis of Sleep Apnea . . . . . . . . . . . . . . 411 Gopal Allada Definitions of Sleep-Disordered Breathing (SDB) . . . . 411 Epidemiology of SDB in the Elderly . . . . 412 Reasons for Sleep Apnea in the Elderly . . . . 412 Taking the History of a Patient with Suspected OSAHS . . . . 413 Clinical Examination of a Patient with Suspected OSAHS . . . . 414 Radiologic Imaging in the Diagnosis of Obstructive Sleep Apnea . . . . 416 Specific Methodologies in the Diagnosis of OSAHS . . . . 417 Unattended Monitoring in Diagnosing OSAHS . . . . 418 Polysomnography in the Evaluation of OSAHS . . . . 421 Recent Reviews and Guidelines . . . . 422 Clinical Decision-Making Algorithm . . . . 425 Summary . . . . 425 References . . . . 425 39. Snoring: Simple to Obstructive Apnea . . . . . . . . . . . . . . . . . . . . 429 Kenny P. Pang, Amy R. Blanchard, and David J. Terris Introduction . . . . 429 Epidemiology . . . . 430 Pathophysiology . . . . 430 Differential Diagnosis . . . . 431 Clinical Examination . . . . 432 Lateral Cephalometry . . . . 433 Diagnosis of SDB . . . . 434 Snoring Treatment . . . . 434 Summary . . . . 435 References . . . . 436 40. Upper Airway Resistance Syndrome . . . . . . . . . . . . . . . . . . . . . 437 Michel A. Cramer Bornemann, Vance D. Bachelder, and Robert H. Maisel Introduction . . . . 437 Definitions in Sleep-Disordered Breathing . . . . 438 Determinants of Upper Airway Patency During Sleep . . . . 438 Sleep in the Elderly . . . . 439 The First Descriptions of UARS . . . . 440 Pathophysiology of UARS . . . . 442

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Clinical Features and Diagnosis of UARS . . . . 443 Sequelae of UARS . . . . 445 Noninvasive Clinical Management . . . . 446 Difficulties in Assessing Surgical Outcomes in UARS . . . . 446 References . . . . 447 41. Surgical Treatment of Sleep-Disordered Breathing . . . . . . . . . . . . 449 Kenny P. Pang, Amy R. Blanchard, and David J. Terris Introduction . . . . 449 Nasal CPAP . . . . 450 Oral Appliances . . . . 450 Surgical Treatment . . . . 451 Conclusions . . . . 456 References . . . . 456 42. Obstructive Sleep Apnea Epidemiology and Patient-Centered Measures . . . . . . . . . . . . . . . . . . . . . . . . . 459 Eric J. Kezirian and Edward M. Weaver Introduction . . . . 459 Definitions of Obstructive Sleep Apnea Syndrome . . . . 459 Prevalence of Obstructive Sleep Apnea Syndrome in Geriatrics . . . . 460 Is Sleep Apnea Different in the Elderly? . . . . 461 Sleep Apnea and Quality of Life . . . . 462 Assessment of Symptoms in Sleep Apnea . . . . 463 Assessment of Sleep Apnea–Related Quality of Life . . . . 463 Geriatric Sleep Apnea and Quality of Life . . . . 463 Conclusions . . . . 466 References . . . . 466 PART VI: HEAD AND NECK PROBLEMS 43. Overview of Head and Neck Cancer . . . . . . . . . . . . . . . . . . . . . 471 Miriam N. Lango and Ara A. Chalian Introduction . . . . 471 Aging and Cancer . . . . 472 Biology of Aging and Cancer . . . . 473 Treatment of the Elderly Head and Neck Cancer Patient . . . . 475 Age, Comorbidity, and Functional Status . . . . 475 Comorbidity . . . . 476 Functional Status . . . . 477 Cognition/Nutrition/Social Support/Polypharmacy . . . . 478 Therapeutic Options for Elderly Head and Neck Cancer Patients . . . . 478 Perioperative Risk Assessment . . . . 479 Incidence of Surgical and Nonsurgical Complications in the Elderly . . . . 479

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Therapeutic Modalities: Functional Outcomes and Quality of Life . . . . 480 References . . . . 480 Annotated Bibliography . . . . 481 44. Head and Neck Cancer in the Geriatric Patient . . . . . . . . . . . . . 483 Jack L. Gluckman and Michael Wolfe Introduction . . . . 483 Evaluation of the Geriatric Patient with Head and Neck Cancer . . . . 484 The Decision-Making Process . . . . 486 Therapeutic Modalities . . . . 486 Patient Follow-Up . . . . 488 Rehabilitation . . . . 489 Conclusion . . . . 489 References . . . . 489 Annotated Bibliography . . . . 490 45. Thyroid Diseases in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . . 491 Maisie Shindo and Frances Tanzella Introduction . . . . 491 Hypothyroidism . . . . 491 Hyperthyroidism . . . . 493 Nontoxic Goiters . . . . 496 Thyroid Malignancies . . . . 498 References . . . . 499 46. Parathyroid Diseases in the Elderly . . . . . . . . . . . . . . . . . . . . . . 501 Maisie Shindo and Frances Tanzella Pathophysiology of Hyperparathyroidism . . . . 501 Diagnosis . . . . 502 Manifestations of Primary Hyperparathyroidism . . . . 502 Evaluation . . . . 504 Surgical Treatment . . . . 504 Nonsurgical Management . . . . 508 Outcome of Treatment . . . . 508 Conclusions . . . . 509 References . . . . 509 47. Facial Nerve Dysfunction: Etiology and Incidence in the Elderly—Acute Management . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Catherine P. Winslow and Mark K. Wax Introduction . . . . 513 Anatomy . . . . 513

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Etiology . . . . 514 Classification . . . . 514 Investigation . . . . 514 Management . . . . 515 References . . . . 519 48. Disorders of the Salivary Glands . . . . . . . . . . . . . . . . . . . . . . . . 521 Karen T. Pitman and Jonas T. Johnson Introduction . . . . 521 Basic Science . . . . 521 History . . . . 522 Physical Examination . . . . 523 Diagnostic Testing . . . . 524 Common Salivary Disorders . . . . 524 Treatment Options . . . . 526 Summary . . . . 527 References . . . . 527 49. Reconstructive Issues in the Elderly Patient with Head and Neck Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529 Eric Genden and Mark K. Wax Introduction . . . . 529 Types of Flaps . . . . 530 Fasciocutaneous Free Tissue . . . . 532 Summary . . . . 536 References . . . . 538 PART VII: APPEARANCE CHANGES 50. Appearance Changes with Aging . . . . . . . . . . . . . . . . . . . . . . . . 541 Donna J. Millay Introduction . . . . 541 Skin Changes . . . . 541 Skin Lesions . . . . 545 Appearance Changes of the Head and Neck . . . . 546 Forehead . . . . 547 Periorbital Region . . . . 549 Midface Region . . . . 550 Nose . . . . 550 Perioral Region . . . . 551 Neck . . . . 551 Ear . . . . 552 Summary . . . . 553 References . . . . 553

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51. Reconstruction in the Elderly Patient . . . . . . . . . . . . . . . . . . . . . 555 Adam T. Ross and Tom D. Wang Introduction . . . . 555 Reconstruction and Depression . . . . 556 Anesthesia . . . . 556 Effects of Aging . . . . 557 Etiology . . . . 557 Preoperative Assessment . . . . 558 General Reconstructive Concepts . . . . 558 Types of Reconstruction . . . . 562 Anatomical Considerations in the Elderly . . . . 570 Trauma and the Elderly Patient . . . . 575 Summary . . . . 577 References . . . . 578 52. Cosmetic Surgery in the Elderly Patient . . . . . . . . . . . . . . . . . . . 581 Oren Friedman, Tom D. Wang, and Ted A. Cook Introduction . . . . 581 The Aging Process . . . . 582 Rejuvenation of the Aging Face . . . . 582 The Aging Face Syndrome . . . . 583 Conclusion . . . . 599 References . . . . 599 PART VIII: PSYCHOSOCIAL ISSUES IN THE CARE OF THE ELDERLY 53. Delirium and Dementia in the Elderly Serge Gauthier Delirium . . . . 603 Dementia . . . . 604 References . . . . 608

. . . . . . . . . . . . . . . . . . . . 603

54. Sensory Impairment and Deprivation in the Elderly . . . . . . . . . . . 609 Allen R. Huang Visual Impairment . . . . 610 Auditory Impairment . . . . 611 Smell and Taste Disturbances . . . . 611 Tactile Impairment . . . . 612 Assessment . . . . 612 Management and Interventions . . . . 613 Conclusions . . . . 615 References . . . . 615

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55. Social Isolation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 617 Vanessa Sakadakis Introduction . . . . 617 Social Isolation and Loneliness . . . . 617 Social Isolation and Elderly People . . . . 618 Assessment and Intervention . . . . 619 Conclusion . . . . 621 References . . . . 621 Annotated Bibliography . . . . 622 56. Substance Misuse in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . 625 David W. Oslin Introduction . . . . 625 General Description of the Clinical Problem . . . . 625 Magnitude of the Problem . . . . 626 Why Is Substance Abuse Particularly Important in the Geriatric Population? . . . . 627 Common Presentations in the Geriatric Population . . . . 628 Physical Exam and Lab Testing . . . . 629 Treatment Options . . . . 630 Summary . . . . 633 References . . . . 633 57. Elder Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635 Mark J. Yaffe Introduction . . . . 635 Definition . . . . 635 Manifestations . . . . 636 Epidemiology . . . . 637 Risk Factors . . . . 638 Mortality Consequences of Elder Abuse . . . . 638 Physician Detection . . . . 639 Elder Abuse Screening . . . . 640 Outcomes from Detection . . . . 640 Summary . . . . 641 References . . . . 641 58. Anxiety and Depression in Older Patients . . . . . . . . . . . . . . . . . 647 Jeffrey P. Staab Introduction . . . . 647 Overview of Anxiety and Depression in the Elderly . . . . 647 Manifestations of Anxiety and Depression in Otorhinolaryngologic Practice . . . . 648

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Association of Anxiety and Depression with Otorhinolaryngologic Illnesses . . . . 650 Head and Neck Surgery . . . . 653 Diagnosis and Treatment of Anxiety and Depression in Otorhinolaryngologic Practice . . . . 654 Treatment Options . . . . 657 Conclusion . . . . 658 References . . . . 659 Annotated Bibliography . . . . 660 59. Coordination of Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 661 Steven C. Zweig Care Coordination . . . . 661 The Future of Care Coordination . . . . 664 References . . . . 664 60. Polypharmacy in Older Adults . . . . . . . . . . . . . . . . . . . . . . . . . 667 Emily R. Hajjar and Joseph T. Hanlon Introduction . . . . 667 Polypharmacy Definitions and Epidemiology . . . . 667 Consequences of Polypharmacy . . . . 668 Principles of Geriatric Pharmacotherapy . . . . 670 Summary . . . . 671 References . . . . 671 PART IX: PERIOPERATIVE ISSUES 61. Preoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 675 Christopher D. Newell Introduction . . . . 675 Cardiovascular System . . . . 675 Pulmonary System . . . . 677 Endocrine System . . . . 677 Renal System . . . . 677 Nervous System . . . . 678 Nutrition . . . . 678 Preoperative Testing . . . . 678 Risk Stratification . . . . 681 Conclusion . . . . 682 References . . . . 682 62. Anesthesia for Otorhinolaryngologic Surgery in the Elderly Myrdalis Dı´az-Ramı´rez Introduction . . . . 685 Physiologic Changes in the Elderly and Common Comorbidities . . . . 686

. . . . . 685

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Contents

Preoperative Evaluation and Treatment . . . . 691 Intraoperative Care . . . . 693 Postoperative Complications . . . . 699 Specific Cases . . . . 699 Conclusion . . . . 701 References . . . . 701 63. Postoperative Care for the Geriatric Patient . . . . . . . . . . . . . . . . 705 Robert L. Cross Jr. and Richard Hanson Introduction . . . . 705 Cardiovascular System . . . . 705 Pulmonary System . . . . 706 Gastrointestinal System . . . . 708 Renal System . . . . 709 Endocrine System . . . . 709 Neurologic System . . . . 710 Conclusion . . . . 712 Bibliography . . . . 712 64. Informed Consent . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 715 Cheryl Ellis Vaiani References . . . . 719 65. Pain Management in the Elderly . . . . . . . . . . . . . . . . . . . . . . . . 721 David Sibell The Size of the Problem . . . . 721 Basic Science of Chronic Pain . . . . 722 Diagnosis of Painful Disorders in the Elderly . . . . 724 Differential Diagnosis . . . . 726 Treatment Options . . . . 728 Interventional Treatments . . . . 733 General Treatment Concepts . . . . 735 References . . . . 735 PART X: END OF LIFE 66. Advance Directives and Do Not Resuscitate Orders . . . . . . . . . . . 739 Cheryl Ellis Vaiani References . . . . 742 67. End-of-Life Issues in the Otolaryngology—Head and Neck Surgery Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . 745 Gerry F. Funk Introduction . . . . 745 HNC Management from a Palliative Care Perspective . . . . 745 Special Considerations in the Terminal Care of HNC Patients . . . . 746

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History and Information Gathering . . . . 747 Physical Examination . . . . 748 Developing a Plan . . . . 749 Specific Management Issues . . . . 750 Summary . . . . 760 Case Studies . . . . 760 References . . . . 762 Annotated Bibliography . . . . 764 68. Hospice for the Otolaryngologist . . . . . . . . . . . . . . . . . . . . . . . . 765 Paul Bascom Historical Perspective . . . . 765 Hospice—A Philosophy of Care and a Program of Services . . . . 766 Discussing Hospice Care with Patients and Families . . . . 767 Index . . . . 771

Contributors

Gopal Allada Division of Pulmonary and Critical Care and Sleep Medicine, Department of Pulmonary and Critical Care, Oregon Health and Science University, Portland, Oregon, U.S.A. Sonia Ancoli-Israel Department of Psychiatry, University of California–San Diego and VA San Diego Healthcare System, San Diego, California, U.S.A. Jonathan E. Aviv Voice and Swallowing Center, Department of Otolaryngology— Head and Neck Surgery, College of Physicians and Surgeons, Columbia University, Columbia University Medical Center, New York–Presbyterian Hospital, New York, New York, U.S.A. Vance D. Bachelder Department of Biomedical Engineering, University of Minnesota Graduate School, Medical Instrumentation and Device (MIND) Laboratory/Bakken Institute, Minneapolis, Minnesota, U.S.A. Steven Barczi Section of Geriatrics, Department of Medicine, University of Wisconsin–Madison, and Geriatric Research Education and Clinical Center, William S. Middleton Veterans Hospital, Madison, Wisconsin, U.S.A. Paul Bascom Palliative Medicine and Comfort Care Team, Division of General Internal Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A. Amy R. Blanchard Section of Pulmonary/Critical Care Medicine, Medical College of Georgia, Augusta, Georgia, U.S.A. Michel A. Cramer Bornemann Departments of Neurology and Pulmonary/Critical Care Medicine, Minnesota Regional Sleep Disorders Center, Hennepin County Medical Center, Minneapolis, Minnesota, U.S.A. Sidney N. Busis Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. Stephen J. Chadwick Department of Otolaryngology, Southern Illinois School of Medicine, Springfield, and ENTA Allergy Head and Neck Institute, Decatur, Illinois, U.S.A. xxix

xxx

Contributors

Ara A. Chalian Geriatric Committee AAO-HNS, Department of Otolaryngology— Head and Neck Surgery, University of Pennsylvania, Philadelphia, Pennsylvania, U.S.A. Marshall Chasin

Musicians’ Clinics of Canada, Toronto, Ontario, Canada

Margaret A. Chen Division of Otolaryngology—Head and Neck Surgery, VA San Diego Healthcare System, and Department of Surgery, UCSD Medical Center, San Diego, California, U.S.A. Daniel H. Coelho Section of Otolaryngology, Yale University School of Medicine, New Haven, Connecticut, U.S.A. Bobby M. Collins Oral Medicine and Pathology, University of Pittsburgh School of Dental Medicine, Pittsburgh, Pennsylvania, U.S.A. Nadine Connor Division of Otolaryngology, Department of Surgery, University of Wisconsin–Madison, Madison, Wisconsin, U.S.A. Ted A. Cook Department of Otolaryngology—Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Robert L. Cross Jr. Department of Anesthesiology and Perioperative Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A. Sam J. Daniel Saliva Management Clinic, McGill University Health Center, Montreal Children’s Hospital, Montreal, Quebec, Canada Stephanie K. Daniels Research Service, VA Medical Center, and Department of Psychiatry and Neurology, Tulane University Health Sciences Center, New Orleans, Louisiana, U.S.A. Terence M. Davidson Division of Otolaryngology—Head and Neck Surgery, VA San Diego Healthcare System, and Department of Surgery, UCSD Medical Center, San Diego, California, U.S.A. Myrdalis Dı´az-Ramı´rez Department of Anesthesiology and Perioperative Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A. Joanne DeLuzio Graduate Department of Speech-Language Pathology, University of Toronto, Toronto, Ontario, Canada Richard L. Doty Smell and Taste Center, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, U.S.A. Umamaheswar Duvvuri Division of Sino-Nasal Disorders and Allergy, Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. David E. Eibling Department of Otolaryngology—Head and Neck Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

Contributors

xxxi

Michelina Fato Department of Medicine, Western Pennsylvania Hospital, Pittsburgh, Pennsylvania, U.S.A. Berrylin J. Ferguson Division of Sino-Nasal Disorders and Allergy, Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. Lavinia Fiorentino Doctoral Program in Clinical Psychology, San Diego State University and University of California–San Diego, San Diego, California, U.S.A. Karen J. Fong Department of Otolaryngology—Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Anne L. Foundas Department of Psychiatry and Neurology, Tulane University Health Sciences Center, and Neurology Section, VA Medical Center, New Orleans, Louisiana, U.S.A. Oren Friedman Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology, Mayo Clinic, Rochester, Minnesota, U.S.A. Gerry F. Funk Department of Otolaryngology—Head and Neck Surgery, University of Iowa College of Medicine, Iowa City, Iowa, U.S.A. Joseph M. Furman Division of Balance Disorders, Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. Serge Gauthier McGill Center for Studies in Aging, McGill University, Montreal, Quebec, Canada Eric Genden Department of Otolaryngology, Mount Sinai Medical Center, New York, New York, U.S.A. Jack L. Gluckman Department of Otolaryngology—Head and Neck Surgery, University of Cincinnati Medical Center, Cincinnati, Ohio, U.S.A. James S. Goodwin Department of Internal Medicine, Sealy Center on Aging, University of Texas Medical Branch, Galveston, Texas, U.S.A. Timothy C. Hain Departments of Physical Therapy, Neurology, and Otolaryngology, Northwestern University Medical School, Chicago, Illinois, U.S.A. Emily R. Hajjar Philadelphia College of Pharmacy, University of the Sciences in Philadelphia, Philadelphia, Pennsylvania, U.S.A. Joseph T. Hanlon Department of Medicine (Geriatrics), University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. Richard Hanson Department of Anesthesiology and Perioperative Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A. Allen R. Huang Geriatric Medicine, McGill University Health Centre, Royal Victoria Hospital, Montreal, Quebec, Canada

xxxii

Contributors

Jonas T. Johnson Department of Otolaryngology and Radiation Oncology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A. Eric J. Kezirian Department of Otolaryngology—Head and Neck Surgery, University of California–San Francisco, San Francisco, California, U.S.A. Horst R. Konrad Division of Otolaryngology—Head and Neck Surgery, Southern Illinois University School of Medicine, Springfield, Illinois, U.S.A. Karen M. Kost Department of Otolaryngology, McGill University, Montreal General Hospital, Montreal, Quebec, Canada Miriam N. Lango Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, U.S.A. Sue Ellen Linville Department of Speech Pathology and Audiology, Marquette University, Milwaukee, Wisconsin, U.S.A. Lianqi Liu Department of Psychiatry, University of California–San Diego and VA San Diego Healthcare System, San Diego, California, U.S.A. Robert H. Maisel Department of Otolaryngology—Head and Neck Surgery, Minnesota Regional Sleep Disorders Center, Hennepin County Medical Center, Minneapolis, Minnesota, U.S.A. Donna J. Millay Division of OtoHNS, Department of Surgery, University of Vermont, Burlington, Vermont, U.S.A. Natasha Mirza Department of Otolaryngology—Head and Neck Surgery, University of Pennsylvania Health System and Philadelphia VA Medical Center, Philadelphia, Pennsylvania, U.S.A. Laura O. Morris Balance and Vestibular Program, Eye and Ear Institute, Centers for Rehab Services, Pittsburgh, Pennsylvania, U.S.A. Thomas Murry Voice and Swallowing Center, Department of Otolaryngology— Head and Neck Surgery, College of Physicians and Surgeons, Columbia University, Columbia University Medical Center, New York–Presbyterian Hospital, New York, New York, U.S.A. Meiho Nakayama Department of Otolaryngology, Aichi Medical University, Aichigun Aichiken, Japan Julian M. Nedzelski Department of Otolaryngology and Head and Neck Surgery, Sunnybrook and Women’s College Health Sciences Centre and University of Toronto, Toronto, Ontario, Canada Christopher D. Newell Department of Anesthesia and Perioperative Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A.

Contributors

xxxiii

Amanda Ortmann Communication Science and Disorders, University of Pittsburgh, and Department of Audiology and Speech Language Pathology, VA Pittsburgh Health Care System, Pittsburgh, Pennsylvania, U.S.A. David W. Oslin Department of Psychiatry, University of Pennsylvania, and Philadelphia VA Medical Center, Philadelphia, Pennsylvania, U.S.A. Catherine V. Palmer Communication Science and Disorders, University of Pittsburgh, and Audiology and Hearing Aids, Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, U.S.A. Kenny P. Pang Department of Otolaryngology, Tan Tock Seng Hospital, Tan Tock Seng, Singapore, and Department of Otolaryngology—Head and Neck Surgery, Medical College of Georgia, Augusta, Georgia, U.S.A. Steven M. Parnes Department of Otolaryngology, Albany Medical Center, Albany, New York, U.S.A. Richard J. Payne Department of Otolaryngology, McGill University, Montreal General Hospital, Montreal, Quebec, Canada Ronen Perez Department of Otolaryngology and Head and Neck Surgery, Sunnybrook and Women’s College Health Sciences Centre and University of Toronto, Toronto, Ontario, Canada Karen T. Pitman Department of Otolaryngology and Communicative Sciences, University of Mississippi Medical School, Jackson, Mississippi, U.S.A. Mukaila A. Raji Department of Internal Medicine, Geriatrics Clinics, Sealy Center on Aging, University of Texas Medical Branch, Galveston, Texas, U.S.A. JoAnne Robbins Sections of Geriatrics and Gastroenterology, Departments of Medicine and Radiology, University of Wisconsin–Madison, and Geriatric Research Education and Clinical Center, William S. Middleton Veterans Hospital, Madison, Wisconsin, U.S.A. Adam T. Ross Division of Facial Plastic and Reconstructive Sugery, Department of Otolaryngology, Medical University of South Carolina, Charleston, South Carolina, U.S.A. Douglas A. Ross Section of Otolaryngology, Yale University School of Medicine, New Haven, Connecticut, U.S.A. Vanessa Sakadakis Social Services Department, McGill University Health Centre, Royal Victoria Hospital, Montreal, Quebec, Canada Clarence T. Sasaki Section of Otolaryngology, Yale University School of Medicine, New Haven, Connecticut, U.S.A.

xxxiv

Contributors

Robert Thayer Sataloff Department of Otolaryngology—Head and Neck Surgery, Thomas Jefferson University and Graduate Hospital, Philadelphia, Pennsylvania, U.S.A. Maisie Shindo Division of Otolaryngology—Head and Neck Surgery, Department of Surgery, State University of New York–Stony Brook, Stony Brook, New York, U.S.A. David Sibell Department of Anesthesiology and Perioperative Medicine, Oregon Health and Science University, Portland, Oregon, U.S.A. Azfar A. Siddiqui School of Dental Medicine, Department of Maxillofacial Prosthetics, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A. Dana S. Smith Department of Otolaryngology/Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Jeffrey P. Staab Departments of Psychiatry and Otorhinolaryngology—Head and Neck Surgery, The Balance Center, University of Pennsylvania Health System, Philadelphia, Pennsylvania, U.S.A. Hinrich Staecker Department of Otolaryngology—Head and Neck Surgery, University of Kansas School of Medicine, Kansas City, Kansas, U.S.A. Frances Tanzella Division of Otolaryngology—Head and Neck Surgery, Department of Surgery, State University of New York–Stony Brook, Stony Brook, New York, U.S.A. David J. Terris Department of Otolaryngology—Head and Neck Surgery, Medical College of Georgia, Augusta, Georgia, U.S.A. Cyrus Torchinsky Division of Otolaryngology—Head and Neck Surgery, VA San Diego Healthcare System, and Department of Surgery, UCSD Medical Center, San Diego, California, U.S.A. Cheryl Ellis Vaiani Institute for the Medical Humanities, University of Texas Medical Branch, Galveston, Texas, U.S.A. Tom D. Wang Department of Otolaryngology—Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Mark K. Wax Department of Otolaryngology/Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Edward M. Weaver Department of Otolaryngology—Head and Neck Surgery, University of Washington, Seattle, Washington, U.S.A. Susan L. Whitney Departments of Physical Therapy and Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

Contributors

xxxv

Catherine P. Winslow Department of Otolaryngology/Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A. Michael Wolfe Department of Otolaryngology—Head and Neck Surgery, University of Cincinnati Medical Center, Cincinnati, Ohio, U.S.A. Dario A. Yacovino Departments of Physical Therapy, Neurology, and Otolaryngology, Northwestern University Medical School, Chicago, Illinois, U.S.A. Mark J. Yaffe Department of Family Medicine, St. Mary’s Hospital Center, McGill University, Montreal, Quebec, Canada Mark A. Zacharek Department of Otolaryngology—Head and Neck Surgery, Henry Ford Hospital, Detroit, Michigan, U.S.A. Steven M. Zeitels Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A. Steven C. Zweig Department of Family and Community Medicine, University of Missouri–Columbia School of Medicine, Columbia, Missouri, U.S.A.

PART I: GERIATRIC OTOLARYNGOLOGY

1 Clinical Approach to the Geriatric Patient Michelina Fato Department of Medicine, Western Pennsylvania Hospital, Pittsburgh, Pennsylvania, U.S.A.

That time of year thou may’st in me behold When yellow leaves, or none, or few, do hang Upon those boughs which shake against the cold— Bare ruin’d choirs where late the sweet birds sang In me thou see’st the twilight of such day As after Sunset fadeth in the West’ Which by and by black night doth take away, Death’s second self, that seals up all in rest. In me thou see’st the glowing of such fire That on the ashes of his youth doth lie, As the death-bed whereon it must expire, Consumed with that which it was nourish’d by. This thou perceiv’st, which makes they love more strong To love that well which thou must leave ere long. William Shakespeare ‘‘Sonnet 73’’

Shakespeare’s beautiful sonnet describes an aging speaker using a series of metaphors such as autumn and twilight. The sonnet allows us to feel sadness for one’s loss of youth and eventual death. The field of geriatrics arouses similar feelings in physicians who care for the elderly, this mind-set often making it difficult for some to work with the elderly. Caring for the aged may remind the practitioner of the inevitability of the practitioner’s own aging and eventual death. The physician may adopt a sense of futility and believe that there is nothing for the physician to offer an elderly patient that will make any difference. Ageism, a form of discrimination, is rampant in our youth-centered culture, and some physicians may just not want to deal with the complex problems that some elderly patients bring with them. Undergraduate and graduate medical education that continues to be inpatient based, with student exposure primarily to severely debilitated and institutionalized elderly, contributes to physician reluctance in dealing with this population as well. 1

2

Fato

However, over the next 20 to 50 years, it will become virtually impossible for any physician in any field (with the obvious exception of pediatric specialties) to avoid contact with an elderly patient. This chapter presents a clinical approach to the geriatric patient. It will help the otolaryngologist—head and neck surgeon—to be less fearful and feel more comfortable with this challenging and complex population, and hopefully even truly enjoy and gain satisfaction from working with these individuals.

DEMOGRAPHICS OF AGING IN AMERICA Throughout human history, the age distribution of the population has resembled a pyramid. Improved and healthier living circumstances have contributed to lower death rates at younger ages. With decreasing fertility rates and decreasing death rates among younger cohorts, it is expected that as these younger cohorts age there will be a ‘‘squaring of the pyramid,’’ (Figs. 1, 2) and that this will be a permanent change in the population. The U.S. population is currently 294 million. Of these, 35 million (nearly 12%) are 65 years and older. This number is expected to double over the next 30 years. This dramatic shift in population demographics over the next three decades will lead to profound social, political, and economic changes in this country and most certainly within our current health care system. The number of Americans aged 80

Figure 1 Age pyramid for humans prior to 1900.

Clinical Approach to the Geriatric Patient

3

Figure 2 Age pyramid of the future.

and older is expected to rise to 32 million in 2050 and the number of centenarians will approach one million by then as well. Patients aged 65 and older accounted for 22% of all office visits to otolaryngologists in the year 2000; this number is projected to double in the next 30 years. All physicians will be required to deal with the effects of this aging population. It is currently estimated that there is a shortage of more than 13,000 physicians needed to care for geriatric patients. It is projected that 35,000 geriatricians will be needed in the United States in 2030; we currently have about 9000. Without substantive changes in U.S. policies regarding medical education, it seems likely that current shortages will increase. Therefore, it is essential that all physicians, including subspecialists, develop a sound clinical approach that takes into account the special needs of this group.

Health Behavior Studies show that older adults expect to be less healthy than younger adults. They also tend to view themselves as more healthy than they are (1). Overestimating their health may result in ‘‘explaining away’’ problems that in fact may be serious. In addition, health care professionals caring for elderly patients often tend to neglect signs and symptoms of disease, often, in fact, reassuring them that frailty and poor health are ‘‘normal’’ accompaniments of aging. Studies show that older persons

4

Fato

tend to underreport symptoms and are more likely to minimize symptoms that are potentially serious (e.g., chest pain, bleeding, dyspnea, depression) (2,3). Reasons that elderly give for not reporting symptoms include ‘‘no big deal,’’ ‘‘nobody cares,’’ ‘‘nothing can be done about it,’’ ‘‘don’t want to bother people’’ (3). In addition, older persons most often attribute symptoms to aging and tend to adopt a wait and watch approach, accepting symptoms as inevitable and delaying seeking health care. To complicate matters, some physicians perceive that older patients have infinite numbers of complaints and may avoid obtaining a thorough history so as not to open ‘‘Pandora’s box.’’ There is no documented support for this and, in fact, younger patients tend to have more hypochondriacal symptoms than the elderly (4,5). Although the elderly underreport symptoms, it is important that physicians recognize that concurrence of disease is common in the elderly and that this multiplicity of problems poses serious risks to their health (6). Detecting treatable disease late in the course of illness results in functional decline and can result in catastrophic illness. Functional decline is the end result for older people, especially for those over age 75, and may be the presenting sign of underlying disease. Elderly patients are less likely to be able to meet their own needs as time passes (7). The so-called geriatric syndromes (falls, confusion, incontinence, dizziness, failure to thrive) are defined as a set of specific functional capacities potentially lost due to a multiplicity of pathologies in multiple organ systems (8). A geriatric syndrome may be an unexpected initial presenting symptom or sign of a classic disease. For example, repeated falls may be the sole manifestation of pneumonia. Because functional loss in an elderly patient is usually a sign of disease, rapid assessment is crucial. Disease in older patients may present in an atypical manner. The elderly may present with minimal or absent symptoms for many conditions associated with wellrecognized symptoms in the younger population. Painless myocardial infarction has been documented in the elderly (9). Hypothyroidism may be more difficult to diagnose due to minimal clinical manifestations. It is important for the clinician to remember that common diseases may present in a nonspecific and atypical manner in the elderly, and that the older patient may underreport symptoms, even if present.

PRACTICAL APPROACH TO THE OFFICE VISIT It must be recognized that the elderly have different needs than younger patients, and that these needs impact the entire spectrum of patient care. The actual environment in which the patient is evaluated may impact successful diagnosis and treatment. Although most commonly evaluation occurs within the physician’s office, in the future it is likely that elderly patients will be evaluated in alternative settings such as the patient’s home, nursing homes, assisted living facilities, rehabilitation centers, hospices, and acute care hospitals. Transportation needs will increasingly become an issue as the number of the elderly continues to rise. Older patients may depend on caregivers, government-funded modes of transportation, and paid drivers (jitney) to get to their appointments. This often leads to late arrivals or missed appointments. It is the physicians’ responsibility to call older patients who miss an appointment if the visit is essential. There may be a multitude of reasons as to why the patient was unable to keep the appointment. In addition, there should be handicap access to parking and to the office. The length of the visit for a geriatric patient may need to be longer than for a younger patient; this will need to be individualized. It may

Clinical Approach to the Geriatric Patient

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take an older patient longer to get into the exam room, undress, sit on an exam table, and much longer to give a history. The physician may also need to review a long list of medications and a complex array of medical problems. In any setting, only about half as much light reaches the retina of elderly patients due to aging changes occurring in the ocular lens. Therefore, brighter lighting is required in both waiting rooms and exam rooms. In addition, any written forms that the patient is required to complete or any patient education materials need to be written in large print to accommodate for decreased vision in the elderly. Elderly patients should also be screened for health literacy (see appendix) as this may affect their ability to read medication labels, fill out forms, or read patient education materials (10). The temperature in the exam room should be between 70 and 80 degrees, since the elderly have increased vulnerability to temperature extremes. Greater than 50% of older patients have presbycusis, with resultant poor speech discrimination, particularly in the presence of background noise. Therefore, all distracting background noise in the examination room such as fans, music, etc., should be eliminated, and the setting should be quiet. The physician should speak in a lower pitched voice and face the patient directly to allow lip reading. Elderly patients often have difficulty arising from a sitting position; hence, chairs with a higher than standard seat, or even a mechanical lift to assist in arising, should be available. If an exam table is used, the office should ideally have a mechanical one that lowers and rises to assist the patient in getting on and off the table. If this is not available, a broad-based stool and handrail should be available for mounting and dismounting the exam table (8). A geriatric patient should never be left alone on the exam table to dismount after an exam. Assistance should be offered to reduce the risk of a fall. In addition, the office setting should be evaluated for fall hazards (loose rugs, exposed cords or wires, slippery floors, etc.) and appropriate remedies taken. Distracting and busy patterns on both walls and carpets should be avoided in both the waiting room and the exam room as this may increase the falls risk.

OBTAINING THE HISTORY When obtaining the history from a geriatric patient, it is important to determine the patient’s reliability as a historian as soon as possible. On the other hand, if a patient carries the diagnosis of dementia, one should not assume that the history is unreliable. Dementia includes a wide spectrum of disorders with differing cognitive ability. Patients may have minor cognitive deficits that would be noted only on extensive neuropsychiatric testing. One should begin the history with some simple questions and preface them by stating ‘‘these are a few questions that I ask of all of my patients.’’ The physician or nurse should then proceed to ask time, place, date, and other orientation questions in addition to the reason for the visit. If the answers are not accurate and there is a concern of cognitive impairment, it is essential that a family member or caregiver accompany the patient to future visits. Regardless, it is still possible to get important information just by listening to the patient. If a family member is present, it is critical that the physician allow the patient to answer questions directly. If the patient is unable to do so, or if the physician needs clarification, only then should the family member be addressed. In addition, it is also important for the physician to be able to access old medical records and to speak with other providers that have cared for the patient.

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Most elderly do not present with one chief complaint, but with multiple complaints that may or may not be inter-related. It is highly unlikely, in fact, that there will be one unifying disease, since most older patients have multiple disorders. This may be less of a problem in the otolaryngologist’s office, since the physician is focusing on one system. However, it is important to keep in mind that all the complaints referable to the head and neck may not be attributable to one problem. The importance of eliciting an adequate medication history cannot be overemphasized. Older patients tend to be on multiple prescribed medications and over-thecounter remedies. Some may be contraindicated and others may counteract each other. In all outpatient settings, it should be emphasized that every patient bring all their medication bottles to every visit. In addition to the prescribed medication bottles, the physician must specifically inquire about herbal and other remedies that are not prescribed. Elderly patients commonly use these, and often do not consider them to be ‘‘medications.’’ If the patient is coming alone or if a family member is going to accompany the patient, they should be instructed to gather all medications in the home and bring them to their visit, including laxatives, vitamins, cold preparations, creams and lotions, herbs, etc. The physician can then review all the medications with the patient and ask about dosing.

SOCIAL HISTORY The perception that the patient’s social history is relevant only to the primary care provider can result in suboptimal specialty care in the elderly. Knowledge of the patient’s living arrangements is essential and crucial to planning care for the patient. It is important to assess whether the patient lives alone, whether family members live nearby, whom they call in case of an emergency, and whether they see any one individual on a daily or regular basis. The availability of a caregiver will often impact on patient compliance with medications and any home care plan. Social isolation is a common problem in the elderly, and is addressed in a dedicated chapter in this text. It is known that individuals with extensive social networks do better in terms of function and have decreased mortality (11). The physician should also inquire about the home environment, particularly the presence of stairs in the home, loose rugs or other fall hazards, adequate heating and cooling, and neighborhood safety (the latter may be assessed by asking ‘‘do you feel safe in your home?’’). It is extremely important to ask the patient about finances as many elderly in this country live at or below the federal poverty level. Questions such as ‘‘what is your monthly income and what is/are the source(s)’’ may be asked on an intake form. It is not unusual for an older person to be living on 400 dollars per month from Social Security. This directly affects both medication and visit compliance. Most insurers require copayments per medication, which can quickly add up, making them unaffordable. In addition, patients on a very limited income may also not be able to afford the physician co-payment.

NUTRITION Undernutrition increases in prevalence with age. However, with the increasing obesity rates in this country, we may see overnutrition as a growing problem among

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elderly patients in the future. Undernutrition is commonly a result of oral or gastrointestinal disorders, medication side effects, psychiatric disease including dementia, inability to shop for or prepare food, lack of companionship, anorexia, poor dentition or ill-fitting dentures, and skipped meals. It is almost always multifactorial in the elderly. A low serum albumin is a good marker of poor nutritional status over the preceding months and correlates with mortality (12). Undernutrition contributes to sarcopenia and frailty in the old.

ALCOHOL, TOBACCO, AND DRUG USE DISORDERS It is important to screen all elderly patients for drug, tobacco, and alcohol use disorders. Alcohol use disorders are not uncommon in the elderly and routine questions, such as in the CAGE questionnaire (see appendix), should be included in the history. Alcoholism may present as falls, depression, or dementia, and may be masked without careful questioning. Tobacco cessation is of benefit to health even beyond the age of 80 (13,14). The author has encountered several patients over the age of 60 who have heroin and/or cocaine addiction. Substance abuse disorders should be considered in all patients and appropriate screening, intervention, treatment, and referral should be done when necessary.

FUNCTIONAL STATUS Patients should directly be asked about their ability to function at home. If there are any questions, screening with the BADL (basic activities of daily living) (see appendix) and IADL (instrumental activities of daily living) (see appendix) instruments should be performed (15,16). These tools may be administered by a registered nurse (RN), licensed practical nurse (LPN), or medical assistant (MA) prior to the physician visit. The BADL and IADL address such issues as mobility, bathing, transferring, continence, hygiene, shopping, cooking, managing money, using the telephone, etc., and are reliable indicators of performance. Loss of function, as measured by these instruments, contributes to a decline in health.

END OF LIFE It is essential to determine individual preferences and values regarding end-of-life care prior to recommending or providing aggressive treatment measures. Often elderly patients are offered aggressive surgery or medical treatment and plans are made only to find out later that these are incongruent with the patient’s goals. All patients should routinely be asked on their first visit, even to a subspecialist, whether they have executed a living will or have a proxy decision maker should they become unable to make health care decisions for themselves. These questions can be part of a routine intake questionnaire used by all patients. Neither of these advance directives requires a notary or an attorney, and standard forms are available through state medical societies. The patient and family can complete these forms together and then provide a copy to the physician. The physician should always respect the patient’s wishes when they are voiced.

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PHYSICAL EXAMINATION Vital signs should always be obtained at every visit; this should include height, weight, BMI or waist circumference, blood pressure sitting and standing, pulse, and temperature. Orthostatic hypotension is common in the elderly and is often multifactorial in origin (e.g., medications, autonomic dysfunction). Screening with a handheld audiometer is also useful in the elderly and can easily be done by an MA. General appearance should take note of dress (e.g., stains), hygiene (odor of urine or stool), youthful or aged appearance, frailty, and grooming. With the elderly patient, a picture is worth a thousand words! Skin changes greatly with age. There is thinning and loss of elasticity. Wrinkling is more prominent in smokers and in individuals with a history of sun exposure. The head and neck area should be examined for actinic keratoses, basal cell carcinomas, and pigmented lesions that may indicate melanoma. Cherry angiomas and seborrheic keratoses, which are benign lesions, are commonly encountered. Head and Neck Examination This exam begins with careful observation of the skin for premalignant and malignant conditions. The temporal arteries should be palpated for pain, nodularity, and pulse. Visual acuity by a pocket Snellen chart held 10 inches from the eye is more practical than the wall chart. The whispered voice is as sensitive as an audioscope for detection of hearing loss (17). The ear canal and drum should be examined using an otoscope; cerumen impaction is a common cause of hearing loss. Oral examination should include palpation and inspection; if dentures are present they should be removed. It is important to examine for sores, oral cancers, and tooth and gum health. The neck examination should include the thyroid, the presence or absence of carotid bruits or other vascular sounds, and palpation for lymphadenopathy. ELDER ABUSE AND NEGLECT Elder abuse and neglect is a serious problem in the United States. It is widespread and affects thousands of vulnerable elderly. However, similar to child abuse and neglect, it is largely hidden under the shroud of family privacy. It is estimated that only about one out of 14 elder abuse cases are reported (18). Studies have indicated that abusers of elderly patients are most often caregivers, employees of facilities, or children of the abused. The most commonly abused are old-old females (mean age 84) in poor physical and mental health. Abuse of an elder person may be physical, psychological, or financial; it may also include neglect or a combination of these. Most states have mandatory reporting laws for elder abuse and neglect by health care providers. The physician should report any suspicions to Adult Protective Services within their local department on aging. All physician offices should screen on the intake questionnaire, asking such questions as ‘‘do you feel safe in your home?’’ It is important to interview an elderly patient alone in the exam room at some point during the history and physical exam. The presence of fresh and healing injuries or a history of repeated falls may suggest abuse; however, it is essential that the physician recognize that most types of abuse are not obvious, especially in the elderly. Dehydration and extensive decubitus ulcers may be evidence of neglect in institutionalized elderly. Neglect may also present in the office setting with poor hygiene, evidence of malnourishment, inappropriate dress, reports of being left in an unsafe situation,

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urine burns, and inability to obtain needed medications. Financial exploitation may manifest itself as unexplained loss of pension or social security checks or loss of personal belongings. Abandonment may present as being ‘‘dropped off’’ at the emergency room with no family available and no intention of a family member returning. It is important that such findings are documented in the medical record. Elder abuse and neglect is a significant problem. Physicians have an extremely important role in assessing, detecting abuse and neglect, and notifying the appropriate authorities (19).

DEPRESSION AND SUICIDE Depression is one of the most common treatable disorders in the elderly and continues to be treated mainly by primary care physicians. The etiology of depression in older patients is usually multifactorial; genetic, biologic, and psychosocial factors all play a role. Strong social support systems may offer protection from late-life depression and facilitate recovery. Physical health has a strong impact on the mental health of older adults. Epidemiologic studies have found that physical health is the strongest predictor of well-being and emotional health in the elderly (20). Therefore, in patients who may be given the diagnosis of advanced head and neck cancer, it is especially important to assess emotional well-being prior to and after the diagnosis has been given. The Geriatric Depression Scale (see appendix) is a screening instrument that can be included in an intake questionnaire and is highly sensitive (100%) (21). Even the simple question ‘‘Do you often feel sad or depressed?’’ has a sensitivity of 83%. Any patient with a positive screen should be referred for further evaluation. Suicide rates in late life are higher than at younger ages. Suicide is one of the top 10 causes of death in people over 65 (22,23). Older white men have the highest rate of any other age and sex group. The prevalence of alcohol use and dependence and poor health status are major risk factors for suicide in late life. All older patients should be screened for depression, especially prior to receiving bad news.

SUMMARY When we ‘‘behold that time of year,’’ to paraphrase Shakespeare, we need the proper skills. Elderly patients have unique health care needs. An approach to geriatric patients must be tailored appropriately to optimize the diagnosis and therapy for specific symptoms and disorders. As the numbers of elderly patients seeking medical care increases significantly over the next several decades, knowledge of the basic principles of geriatric medicine will become essential for all specialists. Optimal care of the geriatric patient will require that every otolaryngologist (with the exception of pediatric otolaryngologists) understand the unique health care needs and goals of the elderly. The author believes that this book will help meet these educational needs.

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APPENDIX INSTRUMENTAL ACTIVITIES OF DAILY LIVING (SELF-RATED VERSION) For each question, circle the points for the answer that best applies to your situation. 1. Can you use the telephone? Without help With some help Completely unable to use the telephone 2. Can you get to places that are out of walking distance? Without help With some help Completely unable to travel unless special arrangements are made 3. Can you go shopping for groceries? Without help With, some help Completely unable to do any shopping 4. Can you prepare your own meals? Without help With some help Completely unable to prepare any meals 5. Can yon do your own house work? Without help With some help Completely unable to do any housework? 6. Can yon do your own handyman work? Without help Without some help Completely unable to do any handyman work? 7. Can you do your own laundry? Without help Without some help Completely unable to do any laundry at all? 8a. Do you take any medicines or use any medications? Yes (If ‘‘yes,’’ answer questions 8b.) No (if ‘‘no,’’ answer question Sc.) 8b. Do you take your own medicine? Without help (in the right doses at the right time) With some help (take medicine if someone prepares it for you and/or reminds you to take it) Completely unable to take own medicine. 8c. If you had to take medicine, could you do it? Without help (in the right doses at the right time) With some help (take medicine if someone prepares it for you and/or reminds you to take it) Completely unable to take own medicine. 9. Can you mange your own money? Without help With some help Completely unable to handle money.

3 2 1 3 2 1 3 2 1 3 2 1 3 2 1 3 2 1 3 2 1

3 2 1 3 2 1 3 2 1

Scoring: The level of BADL dependence is graded via the score. Independent score 0–1. Dependence increases as the score approaches 6. Source: From Ref. 24.

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BASIC ACTIVITIES OF DAILY LIVING Functional Abilities 1. Bathing–sponge bath, tub bath, or shower 0 ¼ no assistance (gets in and out of tub by self) 1 ¼ uses a device to got in or out of the-tub but able to bathe self 2 ¼ requires partial assistance with bathing 3 ¼ full bath, required (unable to bathe) 2. Dressing–includes getting clothes from closet and drawers (under and outer garments and able to use fasteners. 0 ¼ no assistance with getting clothes and dressing self 1 ¼ able to get clothes and get dressed, except for assistance with shoes 2 ¼ receives assistance with getting clothes or getting dressed 3 ¼ requires complete assistance or stays partly or completely undressed 3. Toileting–going to bathroom for bowel and urine elimation, self-cleaning and arranging clothes. 0 ¼ requires no assistance 1 ¼ requires no assistance but utilizes device (cane, walker, wheelchair, bedpan at night, but able to empty in sun.) 2 ¼ receives partial assistance with, going to the bathroom or in cleansing or arranging clothing 3 ¼ receives full assistance or does not go to the bathroom 4. Transfer 0 ¼ moves well in and out of bed and/or chair without assistance 1 ¼ moves well in and out of bed and/or chair with device 2 ¼ moves in and out of bed and/or chair with, assistance 3 ¼ requires full, assistance 5. Continence 0 ¼ controls urination and. bowel, movement completely by self 1 ¼ has occasional ‘‘accidents’’ 2 ¼ supervision helps keep bowel urine control or is incontinent 3 ¼ catheter is used 6. Feeding 0 ¼ able to prepare foods, serve and. feed self without assistance 1 ¼ requires .help in preparation of food but is able to feed self 2 ¼ requires help in preparation of food, cutting of meat, buttering 3 ¼ receives full assistance or is fed partly or completely by tubes. Scoring: The level of BADL dependence is graded via the score. Independent score 0–1. Dependence increases as the score approaches 6. Source: From Ref. 25.

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CAGE QUESTIONNAIRE





Have you ever felt you should Cut down on your drinking? Have people Annoyed you by criticizing your drinking? Have you ever felt bad or Guilty about your drinking? Have you ever had a drink first thing in the morning to steady your nerves or to get rid of a hangover (Eye opener)?

Scoring: Two or more‘‘Yes’’ answers are considered clinically significant. Source: From Ref. 26.

GERIATRIC DEPRESSION SCALE For each question, choose the best answer for how you felt over the past week. 1. Are you basically satisfied with your life? 2. Have you, dropped many of your activities and interest? 3. Do you feel that your life is empty? 4. Do you often get bored? 5. Are you in good spirits most of the time? 6. Are you afraid that something bad is going to happen to you? 7. Do you feel happy most of the time? 8. Do you often feel helpless? 9. Do you prefer to stay at home, rather than going out and doing new things? 10. Do you feel you have more problems with memory than most? 11. Do you think it is wonderful to be alive now? 12. Do you feel pretty worthless the way you are now? 13. Do you feel full of energy? 14. Do you feel that your situation is hopeless? 15. Do you think that most people are better off then you are?

Yes/NO YES/No YES/No YES/No Yes/NO YES/No Yes/NO YES/No YES/No YES/No Yes/NO YES/No Yes/NO YES/No YES/No

Scoring: The scale is scored as follows: 1 point for each response in capital letters. A score of 0 to 5 is normal; a score above 5 suggests depression. Source: From Ref. 27.

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RAPID ESTIMATE OF ADULT LITERACY IN MEDICINE (REALM) (30)

Note: For copies of the REALM kit please contact: Terry Davis, PhD Prevention and Patient Education Project, Department of Medicine LSU Health Sciences Center 1501 Kings Hwy., PO Box 33932 Shreveport, LA 71130-3932 Phone: (318) 675-4585 Source: From Refs. 28, 29.

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REFERENCES 1. Ferraro K. Self-ratings of health among the old and the old-old. J Health Soc Behav 1980; 21:377. 2. Shanas E. The Health of Older People. Cambridge: Harvard University Press, 1961. 3. Brody EM. Tomorrow and tomorrow and tomorrow: toward squaring the suffering curve. In: Gaitz CM, et al., eds. Aging 2000: Our Healthcare Destiny. Vol. II. New York: Springer-Verlag, 1985. 4. Costa PT Jr, McCrae RR. Somatic complaints in males as a function of age and neuroticism: a longitudinal analysis. J Behav Med 1980; 3:245. 5. Stenback A, et al. Illness and health behavior in septuagenarians. Gerontologist 1978; 33:57. 6. Wilson LA, et al. Multiple disorders in the elderly. Lancet 1962; 2:841. 7. Bedsine RW. Geriatric medicine: an overview. Ann Rev Gerontol Geriatr 1980; 1:135. 8. Cassel, Christine K, et al. Geriatric Medicine, An Evidence-Based Approach. 4th ed. New York: Springer-Verlag, 2003. 9. Bayer AJ, et al. Changing presentation of myocardial infarction with increasing old age. J Am Geriatr Soc 1986; 34:263. 10. Wolf MS, Gazmararian JA, Baker DW. Health literacy and functional health status among older adults. Arch Int Med 2005; 165:1946–1952. 11. Berkman LF. Social networks, support, and health: taking the next step forward. Am J Epidemiol 1986; 123:559. 12. Corti M-C, et al. Serum albumin level and physical disability as predictors of mortality in older persons. JAMA 1994; 272:1036–1042. 13. LaCroix AZ, et al. Smoking and mortality among older men and women in three communities. N Engl J Med 1991; 324:1619–1625. 14. Kawachi I, et al. Smoking cessation and decreased risk of stroke in women. JAMA 1993; 269:232–236. 15. Katz S, Downs TD, Crash H, et al. Progress in development of the index of ADL. Gerontologist 1970; 10:20–30. 16. Lawton MP, Brody EM. Assessment of older people: self-maintaining and instrumental activities of daily living. Gerontologist 1969; 9:179–186. 17. Swan IRC, Browning GG. The whispered voice as a screening test for hearing impairment. J R Coll Gen Pract 1985; 35:197. 18. Pillemer K, Finkelhor D. The prevalence of elder abuse. Gerontologist 1988; 28(1):51–57. 19. AMA. Diagnostic and treatment guidelines on elder abuse and neglect. 1992; AA22:92698 20M 11/92. 20. Goldberg EL, Van Natta P, Comstock GW. Depressive symptoms, social networks and social support of elderly women. Am J Epidemiol 1985; 121:448–456. 21. Yasavage JA, Brink TL. Development and validation of a geriatric depression screening scale: a preliminary report. J Psychiatr Res 1983; 17:37–49. 22. Conwell Y. Suicide in elderly patients. In: Schneider LS, Reynolds CF, Lebowitz BD, Friedhoff AJ, eds. Diagnosis and Treatment of Depression in Late Life: Results of the NIH Consensus Development Conference. Washington, DC: American Psychiatric Press, 1994. 23. Murrell SA, Himmelfarb S, Wright K. Prevalence of depression and its correlates in older adults. Am J Epidemiol 1983; 117:173–185. 24. Lawton MP, Brody EM. Assessment of older people: self-maintaining and instrumental activities of daily living. Gerontologist 1969; 9(3):179–186. 25. Katz S, Ford AB, Moskowitz, RW, Jackson BA, Jaffe MW. Studies of illness in the aged. The index of ADL: A standardized measure of biological and psychological function. 26. Mayfield D, McLeod G, Hall P. The CAGE questionnaire: validation of new alcholism screening instrument. Am J Psychiatry 1974; 131(10):1121–1123.

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27. Sheikh JI, Yesavage JA. Geriatric depression scale: recent evidence and development of a shorter version. Clin Gerontol 1986; 5:165–172. 28. Davis TC, Long SW, Jackson RH, et al. Rapid estimate of adult literacy in medicine: a shortened screening instrument. Fam Med 1993; 25:391–395. 29. Rapid estimate of adult literacy in medicine (REALM) http://www.ama.assn. org/ama/pub/category/9913.html.

2 Biology of Aging Mukaila A. Raji Department of Internal Medicine, Geriatrics Clinics, Sealy Center on Aging, University of Texas Medical Branch, Galveston, Texas, U.S.A.

James S. Goodwin Department of Internal Medicine, Sealy Center on Aging, University of Texas Medical Branch, Galveston, Texas, U.S.A.

INTRODUCTION As the U.S. population ages, a better understanding of the biological basis of development and aging is an important step towards reducing frailty and improving disability-free survival. There is a large body of research on what molecular and physiological changes occur with aging and senescence. Aging is characterized by decreased physiologic reserves and increasing prevalence of frailty and diseases (1–3). Prominent among the physiological changes are decline in immune, renal, neurological, and endocrine functions (Table 1) (4–6). The differences in maximal lifespan between different species and in life expectancy in the same species raise a number of questions about the aging process. What are the mechanisms of aging? Are there ways to slow down the aging process? Can we live forever? The answers to these questions may lie in an in-depth understanding of the complex interaction of genetic programs and epigenetic factors that operate over the lifetime of an organism. In essence, nature (genes) and nurture (nongenetic factors such as environment, nutrition, and medical care) control aging phenotypes (1–3,7–9). Nature sets the limit and determines the responses of organisms to extrinsic factors (nurture). Nurture determines the extent to which the organism reaches the maximum of the genetic program. By modulating individuals’ susceptibility to diseases and disability, these nurture–nature interactions determine health-related quality of life and life expectancy. LIFE EXPECTANCY, LIFE QUALITY, AND LIFE SPAN Life expectancy is defined as the age at which half of a specified population dies—the median lifespan for that species. In the Western world, the human life expectancy has increased from between 50 and 55 in the early 1900s to between 75 and 80 in the late 1990s (1,2,10,11). This increased life expectancy and better health-related quality of 17

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Table 1 Physiology of Aging and Clinical Implications Age-related decrease in enzymes (choline acetyl transferase) for acetylcholine synthesis in aging hippocampus Impaired central processing of speech Decrease in olfactory and gustatory function Decrease in muscle mass and increased fat mass Impaired cellular and mucosal immunity, impaired immunosurveillance, and increased formation of autoantibodies Thinning of epidermis, dermis, and subcutaneous fat Decrease in total body water with an increase in body fat Decreased insulin sensitivity Decreased maximal heart rate and beta adrenergic sensitivity Impaired diastolic function Decreased lung elasticity and increased V/Q mismatching Decrease in liver oxidative metabolism Decrease in renal blood flow, tubular function, and glomerular filtration rate

High risk of dementia and delirium

Decreased word recognition High risk for malnutrition Decreased physical functioning Susceptibility to infections and tumors

High risk of decubitus ulcers and poor wound healing post surgery Increased risk of drug toxicity Increased risk of diabetes Increased risk of orthostatic hypotension Increased risk of heart failure and fluid overload High risk for lung infections and postoperative atelectasis High risk for drug toxicity Increased risk for kidney failure and drug toxicity

life reflect major advances made in last century in hygiene, nutrition, vaccination, and discovery of antimicrobial agents (1,11,12). Additionally, better medical care for major diseases (especially heart attacks and cancers) and increasing adoption of healthier lifestyles (such as smoking cessation and exercise) play a major role in recent gains in life expectancy. Yet, with all these advancements, there has been no change in the maximal life span of humans (1,11,12). The maximum life span refers to the ages of the oldest living members of the community, which in humans range from 90 to 125 (1,11,12). The maximum life span varies across species, a variation thought to reflect differences in metabolic rates and body size, among other factors (2,3). The interspecies lifespan differences and the gains in human life expectancy have generated many theories regarding mechanisms of aging. These aging theories, in turn, have generated tremendous interest in the search for potential antiaging interventions. THEORIES OF AGING Theories of aging, summarized in Table 2, can be classified into two general categories: program (nature-determined and genetic) theories and error (nurturedetermined and epigenetic) theories (1,2,7). This classification is convenient but artificial, as the molecular and cellular mechanisms of aging reflect multiple interactions of genetic and nongenetic factors. Genes modulate the ability of organisms to minimize the amount of error resulting from extrinsic and intrinsic stressors (such as free radicals and toxins). In turn, rates of error accumulation in response to stressors modulate the degree to which the organisms reach the maximal lifespan set by the genetic program.

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Table 2 Theories of Aging Program theories Evolutionary theories Disposable soma theory Late-appearing lethal mutation theory Antagonistic pleitropic gene theory Cellular senescence theories Replicative senescence theory Telomere shortening theory Longevity genes and accelerated aging theories Programmed cell death and apoptosis theory Hypothalamus–pituitary–adrenal–ovarian/testicular axis aging Error theories Free radical and mitochondrial dna damage theories Caloric restriction and longevity Mutation theories DNA mutations Advanced glycosylation end products formation Lipid peroxidation and protein oxidation Cross-linking of sugars and collagens Source: From Refs. 1–3, 7–9.

Program Theories Program theories are also known as intrinsic, developmental, or genetic theories of aging (1,2,7). These theories explain aging as largely a manifestation of a predetermined genetic program. According to these theories, there are predetermined periods for the onset of senescence in different cells, organs, and systems that ultimately lead to organismic deaths. These theories suggest the existence of tight control of development and senescence, in ways similar to fetal development, whereby genes are switched on and off at different phases of life. The switching on and off of genes is thought to determine risks of acquisition of diseases, disability, and death. Evidence for these theories is based on data from studies on evolution, cellular senescence and telomere shortening, programmed cell death, longevity-linked genes, and accelerated aging syndromes. Evolutionary Theories These theories posit that aging and senescence processes are set in motion once the organism undergoes stages of development, maturity, and reproduction—the main evolutionary purpose of ‘‘being’’ (1–3,7). In essence, the ‘‘program’’ optimizes reproduction (and child-rearing) at early life by keeping the body resistant to diseases and stemming the onset of frailty. As the reproductive role is accomplished and the body is no longer needed, genes that promote aging phenotypes progressively manifest— an idea aptly summarized in the disposable soma theories (3,13). Other theories based on this argument are: 1. Late-appearing lethal mutation theory—whereby genes whose protein products hasten organismic deaths manifest only after reproduction has been accomplished (3,14). An example is the Huntington’s chorea gene, which

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manifests after the carriers have had chance for procreation, after which— as evolutionary theory goes—the body is no longer needed (15,16). 2. Antagonistic pleitropic gene theory—states that genes (and their protein products) that facilitate survival and reproductive capability at earlier years may at later years heighten the risks of illnesses and death (3,17). For example, androgens are important for optimal male reproductive ability and muscle and bone functions; but at older age, the hormones increase the risks for prostate cancers and atherosclerotic cardiovascular diseases (2). Cellular Senescence Theory This theory states that the aging process reflects a predetermined age of each cell, an idea based on several observations and evidence for: 1. Fixed division time for a given cell—Hayflick and Moorhead’s replicative senescence theory states that cells have a predetermined maximum number of population doublings (2,18). Several genes (such as retinoblastoma, p53, and p21 genes) have been identified as potential modulators of replicative senescence in various organisms (19–21). These genes suppress tumor formation and promote aging phenotypes by controlling the capacity of the cell to enter into the cell division cycle. For example, in cell cultures, fibroblasts have a fixed number of divisions before stopping, such that fibroblasts from the young undergo more divisions than those from the old before proliferative arrest (1,18). Additionally, cells from patients with accelerated aging such as Werner’s syndrome manifest fewer divisions (as well as higher susceptibilities to genomic instability) compared with cells from normal controls of similar age (22,23). 2. Telomere shortenings with cellular division—Telomeres that cover the end of chromosomes protect genes from oxidative damage and fusion with other chromosomes (24). The telomeres shorten with each cell division. In germ cells and other cells that proliferate throughout life, the enzyme telomerase makes new telomeres and helps maintain a relatively constant telomere length (1,24). Further evidence for the role of telomeres comes from observations that some cancer cells exhibit excessive telomerase activity (1,25). Additional evidence also comes from the association of experimental lengthening of telomeres with an increase in the replicative life spans of those cells (26,27). Longevity Genes and Accelerated Aging Syndromes Evidence for longevity genes comes from several observations: aggregation of extended longevity in siblings of centenarians, clustering of certain genes in longestliving individuals, and the existence of patients with accelerated aging syndromes (8,9,28–30). These longevity genes appear to delay the onset of disease and age-related physiological decline. Thus, many centenarians remain active and independent, with delay of onset of diseases to the very last months of their life—a phenomenon referred to as compression of morbidity (31). Examples of these longevity genes are possession of apolipoprotein E 2 alleles (as opposed to E 4 alleles) and polymorphisms of the angiotensin converting enzyme genes (Refs. 28, 29, 30 and others). Conversely, evidence for aging-promoting genes comes from the premature aging phenotypes found in patients with accelerated aging syndromes (Werner’s and Hutchinson–Gilford syndromes—progeria).

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These patients exhibit early onset of arteriosclerosis, wrinkled skin, and early menopause, among other features (1,23). The majority of these patients die before the age of 50. Programmed Cell Death (Apoptosis) Theory This theory states that cellular senescence and death reflect a ‘‘death’’ program that is triggered in response to some internal or external stimulus (1,7,32). This apoptotic program involves gradual disintegration of cells, condensation of both nuclear and cytoplasmic materials, fragmentation of these materials, and subsequent phagocytosis by nearby cells (1,33). Several genes are involved in programmed cell death including bcl-2 and bad genes, among others (1,7). One way by which programmed cell death modulates aging phenotypes is through age-related apoptosis of cells subserving neuroimmunoendocrine functions. For example, with loss of hypothalamic neurons, there is progressive dysfunction in the hypothalamus–pituitary–adrenal–gonadal axis leading to aging phenotypes of osteoporosis, menopause, and sarcopenia. Similarly, programmed death of immune cells is associated with increased production of autoantibodies, decreased immunosurveillance, lower resistance to infections, and suboptimal free-radical scavenging and DNA repair capacity (1,2,7). Error Theories Error theories are also known as stochastic, extrinsic, or epigenetic theories (1,2,7). These theories explain the aging process and phenotypes as due to random accumulation of errors (such as mutations, damaged DNA, abnormally glycosylated proteins, and X-linked collagen, among others). These errors are thought to account for subsequent dysfunctions at molecular, cellular, and systemic levels. These dysfunctions ultimately lead to senescence and organismic deaths. According to these theories, the errors can occur from exposure to internal and external factors such as infectious agents, ultraviolet radiation, toxins, and free radicals (1,2,7). Error formations by these factors are presumed to be random— stochastic—and outside of genetic control—epigenetic. The main error theories are: Free Radical/Mitochondrial DNA Damage Theories. They state that aging is in part due to progressive damage to vital structures (such as mitochondrial DNA) by reactive oxygen species (free radicals) (8,9,34–36). Free radicals are molecules with unpaired electrons and—by taking electrons from nearby molecules—they readily oxidize nearby cells and tissues. In particular, the oxidation of mitochondrial DNA leads to excessive generation of more free radicals in the respiratory chain cycle, causing more DNA oxidative damage, and the vicious cycle of continued free-radical generation continues. There are several free-radical scavengers (antioxidants) including super oxide dismutase, catalase, ascorbic acid, vitamin E, and glutathione S-transferase (GSTMI) (8,9,37). Thus, these theories posit that aging starts with diminishing capacity of the organisms to remove free radicals and to repair damaged tissues. Evidence for the free-radical theories comes from mutations of antioxidant capacity experiments. In fruit flies and C. elegans worms, mutations that increase the superoxide dismutase activity (an antioxidant enzyme)

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extend the survival of these organisms (8,9,38,39). A study showed that having a specific genetic variant of the antioxidant enzyme was associated with better survival of cochlear cells in aged humans exposed to oxidative noise stress (37). Other evidence supporting the free-radical theory comes from the salutary effect of caloric (dietary) restriction on longevity in insects, worms, and rats (40,41). Calorie restriction means having a 30% to 40% reduction of calories in diets that are otherwise balanced in proteins, carbohydrates, fats, and vitamins. Several mechanisms have been proposed to explain how lower caloric intake delays aging. These mechanisms include reduction of metabolic rates, decreased free-radical generation, and decreased oxidative damage to mitochondrial DNA and other organelles (1,2,7,34). The diet-restrictedorganisms exhibit delayed onset of several age-related physiological declines. The organisms show delayed decline in renal, cardiovascular, immune, and reproductive functions, delayed onset of cancers and osteoporosis, an increase in HDL, and a slow decline of dehydroepiandrosterone (1,2,7,41,42). Mutation Theories. This theory attributes aging to random accumulations of genetic mutations (43–45). These mutations are thought to cause dysfunction in DNA transcription and RNA translation, and abnormal post-translational changes in proteins, lipids, and collagens. Over time, damaged DNA is associated with higher risks of malignant transformation. As well, malformed and malfunctioning proteins accumulate, leading to tissue and system dysfunctions. One mechanism for dysfunctional proteins is through increased formation of advanced glycosylation end products, which have been implicated in cataract formation and loss of auditory hair cells (1,36,46). Onset and pace of the aging process depend on the capacity of organisms for DNA repair, for apoptosis of damaged cells to prevent neoplastic transformation, and for removal of altered proteins. Lipid peroxidation, protein oxidation, and sugar and collagen crosslinking lead to compromised function in the neuroendocrine, immune and other systems. Thus, these physiological dysfunctions are thought to be important mechanisms for the aging process (1,2,7). The dysfunctions contribute to aging phenotypes in part by increasing the susceptibility of the aging animals to age-associated diseases: coronary arteriosclerosis, diabetes, Alzheimer’s disease, cancers, and infections.

ANTIAGING INTERVENTIONS—INSIGHT AND POTENTIALS FROM AGING THEORIES Approaches to slow down the development of aging phenotypes include diet restriction, use of antioxidants, and gene manipulations. To date, no data support extension of human lifespan by caloric restriction or usage of antioxidants, despite strong evidence in other species. On the other hand, better living conditions along with advances in nutrition, infection treatments, and medical care for major geriatric illnesses have contributed to the compression of morbidity to the very end of life, thus allowing for a longer lifetime of independent living.

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SUMMARY AND CONCLUSION The aging process is multifactorial, and no single theory can fully account for aging mechanisms. The aging process reflects the complexity of interactions between genes and the environment, and the adaptive capacity of organisms to respond to new challenges. Genetic programs set the limit of maximal life span for the organism. Reaching this limit depends on exposure to error-inducing intrinsic and extrinsic factors, and the organism’s capacity to moderate these errors and to repair damaged cells. REFERENCES 1. Troen BR. The biology of aging. Mount Sinai. J Med 2003; 70:3–22. 2. Weinert BT, Timiras PS. Physiology of aging. Invited review: theories of aging. J Appl Physiol 2003; 95:1706–1716. 3. Kirkwood TBL, Austad S. Why do we age? Nature 2000; 48:233–238. 4. Naranjo CA, Herrmann N, Mittmann N, Bremner KE. Recent advances in geriatric psychopharmacology. Drugs Aging 1995; 7:184–202. 5. Montamat SC, Cusack BJ, Vestal RE. Management of drug therapy in the elderly. NEJM 1989; 321:303–309. 6. Rochon PA, Gurwitz JH. Drug therapy. Lancet 1995; 346:32–36. 7. Semsei I. On the nature of aging. Mech Ageing Dev 2000; 117:93–108. 8. Robert L, Labat-Robert J. The mechanisms of aging. From genetic to epigenetic. Presse Med 2003; 32:605–614. 9. Knight JA. The biochemistry of aging. Adv Clin Chem 2000; 35:1–62. 10. Oeppen J, Vaupel JW. Broken limits to life expectancy. Science 2002; 296:1029–1031. 11. Olshansky SJ. The demography of aging. In: Cassel KC, Cohen HJ, Larson EB, et al., eds. Geriatric medicine. 3rd ed. New York: Springer-Verlag, 1997:29–36. 12. Hayflick L. The future of ageing. Nature 2000; 408:267–269. 13. Kirkwood TBL. Evolution of ageing. Nature 1977; 270:301–304. 14. Martin GM, Austad SN, Johnson TE. Genetic analysis of aging: role of oxidative damage and environmental stresses. Nat Genet 1996; 13:25–34. 15. Haldane JBS. New paths in genetics. London: Allen & Unwin, 1941. 16. Medawar PB. An unsolved problem of biology. London: Lewis, 1952. 17. Williams GC. Pleitropy, natural selection and the evolution of senescence. Evolution 1957; 11:398–411. 18. Hayflick L, Moorhead PS. The limited in vitro lifetime of human diploid cell strains. Exp Cell Res 1965; 37:614–636. 19. Atadja P, Wong H, Garkavtsev I, et al. Increased activity of p53 in senescing fibroblasts. Proc Natl Acad Sci USA 1995; 92:8348–8352. 20. Stein GH, Beeson M, Gordon L. Failure to phosphorylate the retinoblastoma gene product in senescent human fibroblasts. Science 1990; 249:666–699. 21. Shay JW, Pereira-Smith OM, Wright WE. A role for both RB and p53 in the regulation of human senescence. Exp Cell Res 1991; 196:33–39. 22. Brown WT. Genetic diseases of premature aging as models of senescence. Annu Rev Gerontol Geriatr 1990; 10:23–42. 23. Martin GM, Oshima J. Lessons from human progeroid syndromes. Nature 2000; 408: 262–266. 24. Harley CB, Futcher AB, Greider CW. Telomeres shorten during ageing of human fibroblasts. Nature 1990; 345:458–460. 25. Counter CM, Hirte HW, Baccheti S, Harley CB. Telomerase activity in human ovarian carcinoma. Proc Natl Acad Sci USA 1994; 91:2900–2904. 26. Bodnar AG, Oulette M, Frolkis M, et al. Extension of lifespan by introduction of telomerase into normal human cells. Science 1998; 279:349–352.

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27. Wright WE, Brasiskyte D, Piatyszek MA, Shay JW. Experimental elongation of telomeres extends the lifespan of immortal x normal cell hybrids. EMBO J 1996; 15: 1734–1741. 28. Jazwinski SM. Longevity, genes, and aging. Science 1996; 273:54–59. 29. Schachter F, Faure-Delanef L, Guenot F, et al. Genetic associations with human longevity at the APOE and ACE loci. Nat Genet 1994; 6:29–32. 30. Perls T, Levenson R, Regan M, Puca A. What does it take to live to 100? Mech Ageing Dev 2002; 123:231–242. 31. Fries JF. Measuring and monitoring success in compressing morbidity. Ann Intern Med 2003; 139:455–459. 32. Lockshin RA, Zakeri ZF. Programmed cell death: new thoughts and relevance to aging. J Gerontol 1990; 45:B135–B140. 33. Ellis RE, Yuan JY, Horvitz HR. Mechanisms and functions of cell death. Annu Rev Cell Dev Biol 1991; 7:663–698. 34. Mandavilli BS, Santos JH, Van Houten B. Mitochondrial DNA repair and aging. Mutat Res 2002; 509:127–151. 35. Harman D. Aging: a theory based on free radical and radiation chemistry. J Gerontol 1957; 2:298–300. 36. Levine RL, Stadtman ER. Oxidative modification of proteins during aging. Exp Gerontol 2001; 36:1495–1502. 37. Rabinowitz PM, Pierce Wise J Sr, Hur Mobo B, Antonucci PG, Powell C, Slade M. Antioxidant status and hearing function in noise-exposed workers. Hear Res 2002; 173:164–171. 38. Tower J. Transgenic methods for increasing Drosophila lifespan. Mech Ageing Dev 2000; 118:1–14. 39. Melov S, Ravenscroft J, Malik S, et al. Extension of lifespan with superoxide dismutase/ catalase mimetics. Science 2000; 289:1567–1569. 40. Weindruch R, Walford RL. Dietary restriction in mice beginning at 1 year of age: effect on lifespan and spontaneous cancer incidence. Science 1982; 215:1415–1418. 41. Masoro EJ. Dietary restriction and aging. J Am Geriatr Soc 1993; 41:994–999. 42. Lane MA, Ingram DK, Ball SS, Roth GS. Dehydroepiandrosterone sulfate: a biomarker of primate aging slowed by calorie restriction. J Clin Endocrinol Metab 1997; 82:2093–2096. 43. Strehler BL, Freeman MR. Randomness, redundancy and repair: role and relevance to biological aging. Mech Ageing Dev 1980; 14:15–38. 44. Verzar F. The stages and consequences of aging of collagen. Gerontologia 1969; 15:233–239. 45. Cerami A. Hypothesis: glucose as a mediator of aging. J Am Geriatr Soc 1985; 33:626–634. 46. Ulrich P, Cerami A. Protein glycation, diabetes, and aging. Recent Prog Horm Res 2001; 56:1–21.

3 Introduction to Geriatric Otolaryngology Hinrich Staecker Department of Otolaryngology—Head and Neck Surgery, University of Kansas School of Medicine, Kansas City, Kansas, U.S.A.

INTRODUCTION Otolaryngology as a specialty that deals with a wide variety of disorders that span from communication disorders to allergies and sinusitis to treatment of complex head and neck malignancies. Depending on the practice surveyed, up to one-third of patients seen by the average otolaryngologist are over the age of 65. With an aging population, health care of the elderly is becoming increasingly important. The importance of the older patient has been only partially recognized. Current otolaryngology texts do feature a section devoted to the care of the elderly patient. However, principles of geriatric medicine and issues of concern specific to geriatric patients in the realm of otolaryngology have not been widely applied. Furthermore, a significant portion of available literature dealing with geriatric issues in otolaryngology consists of case reports and case series. The purpose of this review is to assess the state-of-the-art knowledge for otolaryngologic care of the elderly and define important areas for future study in the care of the elderly.

THE AUDITORY SYSTEM Epidemiology The most common otolaryngologic disability affecting the elderly is hearing loss. Our understanding of age-related hearing degeneration has increased significantly through several large population-based studies. A wide variety of age-specific changes as well as the common otologic diseases occur in the elderly. Among the best-characterized populations that have been longitudinally studied is the Framingham cohort. A study of 1662 patients aged 60 to 90 showed an age-related increase in pure tone thresholds and a concurrent decrease in speech discrimination. The rate of hearing decline was found to be equal in men and women, but on average, men started with worse hearing. Interestingly, there was also a slight change in contralateral acoustic reflex thresholds. A further observation of the study was that only 10% of patients who were candidates for hearing aids used them (1). The rate of hearing decline has also been estimated by following a clinic population over a six-year period. Having the measurements taken 25

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by the same audiologist reduced variability in results. In a study of 1475 patients over this six-year period, an average decline in hearing of 1–8 dB was seen at 250 Hz, while a 10–15 dB decline in hearing was noted at 8 kHz. There appeared to be two main patterns of hearing degeneration. The low-frequency pattern of hearing loss appeared to be age dependent and women had worse thresholds than men. The high-frequency pattern of hearing loss had a different pattern of progression; the rate of threshold change decreased with age. This difference in pattern is interpreted as possibly representing a disorder of the stria vascularis (the organ that generates the endocochlear potential) for the low-frequency loss and a hair cell disorder for the high-frequency loss (2). The Baltimore Longitudinal Study of Aging has examined 681 men and 416 women using standard pure tone auditometry from 1965 to present. The individuals were screened for prior otologic disease and noise exposure. This study demonstrated that the rate of loss in hearing sensitivity was greater in women than in men. In men, there was a decrease in hearing sensitivity that was detectable from age 30 on. In terms of gender differences, women tended to have better highfrequency hearing than men did and men had better low-frequency hearing. Overall, there was significant variation within the study group. A conclusion of this study was that there was an age-related decline in hearing (with the aforementioned gender factors) that was present in individuals without any history of noise exposure or evidence of noise exposure on their audiograms (3). This differs somewhat from the interpretation of the Framingham data, which suggested that the preponderance of male high-frequency hearing loss is related to occupational noise exposure. Epidemiological studies have consistently shown a 30% to 70% incidence of age-related hearing loss with widely variable assessment of the degree of impairment induced by the hearing loss. Overall, the degree of hearing loss increases with increasing age and is more prevalent in geriatric patients who are institutionalized (4,5).

Diseases of the Pinna and External Auditory Canal Cerumen impaction can have a significant effect on the hearing of elderly patients. In a random sampling of hospitalized elderly patients over a one-year period, 30% were found to have cerumen impaction. Improved hearing was obtained in 75% of ears that underwent removal of cerumen (6).

Diseases of the Tympanic Membrane and Middle Ear Tympanic Membrane Perforations Numerous studies have examined the age-related risk of otologic surgery both in terms of complications and surgical outcome. Age over 65 has no impact on graft take rate in tympanoplasty (7). In a study of 42 elderly patients (age > 65) with otosclerosis, Vartiainen (8) determined that there was no significant increase in surgical complications and recovery of hearing was similar to that of 275 younger adult patients. Therefore, chronological age is not a contraindication to middle ear surgery either in terms of complications or in terms of outcomes of the surgery. No data comparing quality of life after surgery versus hearing aid placement are available. Also, there have been no controlled intervention trials to establish the best treatment of otosclerosis in older people.

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Eustachian Tube Dysfunction (ETD) ETD has been described in the elderly population but few studies have attempted to establish a clear etiology. When 36 temporal bones of younger adult and elderly patients were examined, calcifications of the eustachian tube cartilage and atrophy of the tensor veli palitini were found to increase in prevalence and severity with age (9). In another study, functional compliance was also found to change with aging, therefore affecting the overall function of the eustachian tube (10). No studies have examined the prevalence of serous otitis media in the elderly, and the contribution of ETD to hearing loss in older people has not been defined.

Presbycusis/Sensorineural Hearing Loss The incidence and impact of age-related sensorineural hearing loss is well established. Recent studies are beginning to define deficits in addition to the previously described degeneration of the auditory hair cells, auditory neurons, and stria vascularis. Schuknecht and Gacek (11) classified presbycusis based on histologic criteria. In the authors’ last review of the subject, they reported that the four diagnostic criteria of age-related hearing loss (sensory cell degeneration, neural degeneration, strial atrophy, and cochlear conductive loss) held up in a review of 21 cases that met the clinical diagnosis of presbycusis. Of note was the observation that most cases seemed to have a mixed pathological pattern. More recent analysis of temporal bone specimens has shown a high incidence of mutations of mitochondrial DNA within the peripheral auditory system (12). Age-related changes in the central nervous system (central presbycusis) also play an important role in hearing deficits in older patients. There are clearly documented auditory brainstem response (ABR) changes that occur with aging. Standard ABR examines the auditory pathway from the cochlea to the inferior colliculus. In a study of 92 subjects aged 50 to 90, and 30 control subjects aged 20 to 29, there was a progressive delay in wave I, wave III, and wave V of the ABR. Interestingly, there was a lengthening in I–V and II–V interpeak intervals observed in the cohort of 70- to 79-year-olds that was not present in the oldest subjects (13). This may suggest that there is a cohort of the very aged that has better than average central (brainstem level) auditory processing. Auditory brainstem response and a central auditory test battery were used to study patients matched for peripheral hearing loss. Patients classified as having a retrocochlear loss (by ABR criteria) overall were poorer performers on the central auditory test battery (14). A number of significant changes occur in the central processing of auditory information in elderly patients. In a study that compared a cohort of young (average age of 26) with a group of older (average age of 70) men, monaural and binaural temporal thresholds were measured. The elderly group showed poorer performance on both monaural and binaural processing tasks. These measurements were independent of peripheral system disease (15). Stach et al. (16) investigated the prevalence of central presbycusis in a study of 700 patients aged 50 and older. One hundred consecutive patients from each fiveyear cohort with a complaint of hearing loss were enrolled and studied for the effects of central auditory processing on hearing. There was an age-related increase in central presbycusis that reached an incidence of 95% by the eighth decade of life. The results were controlled for absolute hearing threshold. This study also examined an age-matched ‘‘nonclinical’’ group (i.e., individuals without complaints

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of hearing loss). This population also demonstrated the presence of central auditory processing disorders, but at a lower rate. A study of 25 normal hearing, cognitively intact adults revealed that central auditory processing declined despite normal cognition, peripheral hearing sensitivity, and linguistic capability. The synthetic sentence identification–ipsilateral competing message test appeared to be the most sensitive measure of central auditory dysfunction (17). There are no clear data on the significance or the functional impact of these findings. Impact of Presbycusis on Quality of Life A number of studies have examined the impact of hearing loss on the elderly. A patient-based outcomes study was carried out on 2466 patients between the ages of 17 and 80. Patients were tested with standard audiologic measures as well as sentences in noise. There was an age-related decrease in performance of auditory tasks such as sentence identification that correlated with increasing hearing thresholds. There was also a matched increase in disability outcomes as measured by validated subjective outcome tools that identified features of communication disability that led to feelings of isolation and depression (13). In a study of over 1800 nursing home residents, sensory deprivation due to hearing loss was found to have a significant impact on social interaction. This was compounded when there was associated visual loss (18). Studies carried out on a cohort of 472 patients with mild to moderate hearing loss with disease-specific outcome measures revealed that even mild hearing losses had a significant impact on perceived emotional, social, and communication function. Sixty-six percent of individuals tested found that this represented a severe handicap for them despite hearing losses ranging from only 25–55 dB (19). In a follow-up study, 194 elderly patients were randomized into two groups, one receiving hearing aids immediately and one being placed on a waiting list. The effect of hearing aid use on quality of life was documented by a variety of outcome tools. There was a statistically significant effect on quality of life and cognitive function demonstrated in the patients that received treatment immediately (20). A study on a population of over 1100 elderly found that hearing loss correlated with depression and decreased independence (21,22). A small (n ¼ 100) case control study of normal subjects and patients with Alzheimer’s disease did show an increase in the odds of having dementia with increasing hearing loss. Increasing degree of hearing loss, however, correlated with increased cognitive impairment in this population (23). There is some evidence to suggest that more accurate assessments of impairment can be derived from the patient’s spouse and that discrepancies between hearing level and perceived deficit may be explained by the presence of central auditory processing deficits (24). Another interesting observation is that hearing loss in the elderly may be associated with depression. In a study of 43 geriatric patients with major depression, age of onset of depression correlated with decreased acuity of hearing. It was unclear from this study if the associated hearing loss could be purely related to increased age of the patient, but it does suggest that the association between hearing loss and depression in the elderly should be investigated (25). Inheritance of Presbycusis The inheritance patterns of presbycusis were studied in the Framingham cohort. Hearing levels in unrelated spouse pairs were compared to sibling pairs and parent– child pairs. Pure tone averages for low-, middle-, and high-frequency hearing

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were calculated to generate an audiometric pattern that was graded as normal, flat loss (consistent with strial atrophy), and high-frequency loss (consistent with sensory pathology). Hearing levels were correlated between the aforementioned groups. There was a grouping of hearing threshold changes within parent–child groups and within sibling groups. Sisters and mother–daughter and mother–son pairs showed an association of hearing threshold changes at all three frequency sets. The strial pattern of loss showed stronger aggregation and association among female relatives. The sensory pattern of hearing loss was found to aggregate in all related pairs except father–child pairs. This was interpreted as showing that there was a stronger heritable component to the strial pattern of sensory loss and that the inheritance of presbycusis had a genetic component in women and a mixed etiology in men (26). The Framingham study also suggested that patients with a history of noise exposure continue to be at greater risk of progression of hearing loss. This may offer an opportunity for intervention (27). The Impact of Hearing Loss on Dementia A number of studies have suggested that there is a link or at least an association between hearing loss and dementia (28). In a study of 30 patients with Alzheimer’s disease and 22 patients with generalized cognitive impairment that presented to a memory disorders clinic, 98% of patients failed a pure tone hearing test (29). This indicates that certain selected populations may have extremely high rates of hearing loss. Gates et al. (30) examined hearing in a cohort of 82 elderly patients enrolled in a prospective Alzheimer’s disease research program. Forty patients were judged to be non-demented based on the clinical dementia rating scale, and 42 were judged to have probable Alzheimer’s disease based on clinical dementia rating scale scores. Pure tone testing showed no difference in the incidence of hearing loss in the two groups, but the probable Alzheimer’s disease group had a much higher incidence of central auditory processing abnormalities. In a follow-up study, Gates et al. (30) examined patients from the Framingham study and found that hearing loss significantly lowered performance on the verbal portions of the mini mental state test. Patients were also tested with synthetic sentence identification with ipsilateral competing message (a test for central auditory dysfunction). A poor score in both ears was associated with a high relative risk of subsequent clinical dementia. This suggests that central auditory processing dysfunction may precede or be an early sign of some dementia. Treatment of Presbycusis It is well accepted that hearing aids are the treatment of choice for the moderately hearing impaired. The choice of hearing aids in the elderly needs to be carefully considered. In a study of in-the-canal (ITC) hearing aid use in 220 patients with an average age of 69, there was significant benefit from amplification. Yet elderly patients found ITC aids difficult to manipulate (31). It is important to take into account central processing as well as cognitive impairment in assessing the impact of these devices on the aging listener. Currently, no hearing aids provide clear transmission of sound in a noisy background, and furthermore, they do little to improve the function of persons with poor speech discrimination. Despite the demonstrated impact of hearing loss on quality of life, only 14% of the elderly with hearing loss are fitted and use hearing aids. No clearly identifiable

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variable (i.e., age, degree of hearing loss, educational status, financial status) has been identified that consistently correlates with hearing aid use (19,20). Newer implantable hearing aids may help with problems of high-frequency gain and feedback, whereas cochlear implantation is beneficial to patients with very poor speech discrimination. Additional ways of improving the function of the hearing impaired is the use of assistive listening devices. Amplified doorbells and phones as well as personal amplification (headphones) for television viewing can improve the function of both the independent and non-independent elderly. For patients with profound hearing loss, cochlear implantation has become the treatment of choice. Shin et al. (32) examined the complication rates and outcomes in 27 patients older than 60 compared to a group of younger patients. This retrospective study as well as several more recent studies showed that there was no increased incidence in complications and that outcome in auditory function was not statistically different between the two groups (33,34). Some studies have also focused on quality-of-life and cost–benefit analysis after cochlear implantation in older patients and overall have concluded the procedure to be a cost-effective and beneficial intervention (35). Neurotology There are a limited number of papers devoted to neurotologic surgery in the elderly. Age was not found to be predictive of return to premorbid function in a series of Meniere’s patients treated with labyrinthectomy (36). There are no large series of elderly patients treated with gentamicin for Meniere’s disease that have been studied. Treatment of acoustic neuroma has been rapidly evolving over the last 10 years. Currently, age is not considered a contraindication for surgical management of the tumor. Some studies do, however, suggest that patient age may affect postoperative complications to some degree. Age >55 was found to be a statistically significant risk factor for the development of postoperative disequilibrium (7). A recent series of papers has examined the growth rate of acoustic neuroma. This has resulted in the observation that a selected group of patients may be candidates for observation rather than surgical excision or radiation. Current studies suggest that in growing tumors (30–40%), the average growth rate is 1 mm/yr, making no treatment other than observation with serial MRI possible in a selected patient population (37,38). De Cruiz et al. (39) examined the quality of life in postoperative acoustic neuroma patients. There was no statistically significant effect of age on patient-based outcome data. Summary Otologic disease, especially age-related hearing loss, is probably the most common otolaryngologic disorder afflicting the elderly. Treatment of presbycusis is currently limited to amplification. The overall low level of use and dissatisfaction with hearing aids is not thoroughly addressed in the literature. Review of the current literature reveals that there are no age-related contraindications to middle ear surgery. There are no significant data available for neurotologic surgery. Recent developments in acoustic neuroma have shown that observation may be a reasonable choice in non-growing tumors in the elderly. The last 10 years have shown that central auditory processing disorders, distinct from peripheral auditory degeneration, affect the hearing of the elderly. It is unclear at present what the impact of this disorder is on communication in the

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elderly. Advances are being made in the understanding of peripheral age-related auditory dysfunction with large cohort studies identifying both genetic and environmental factors. Thus far, even though there have been tremendous advances in molecular biology of presbycusis in animal models, no human ‘‘presbycusis’’ genes have been identified.

VESTIBULAR SYSTEM Balance disorders, though probably as common as auditory problems in the elderly, are complex and have not been as fully studied. A large percentage of balance disorders in the elderly can be attributed to cardiovascular disease, neurologic disease, or medication effects. Most studies on patients with peripheral balance disorders have already had this population selected out. Histological Studies Recent archival temporal bone studies have shown that there is an age-related decline in both vestibular sensory and ganglion cells. Type I hair cells show a significant decline in the cristae, whereas type II hair cells are lost in both the cristae and the macular organs (40). There is also a decline of vestibular ganglion cells with age (41). In a histological study of human brainstems, Alvarez et al. (42) demonstrated that there was an age-related loss of neurons in the descending medial and lateral vestibular nuclei. The neurons of the superior vestibular nucleus were preserved (42). Etiology of Dizziness In a study by Davis (43), the etiology of dizziness in 117 consecutive patients (age > 50) was determined. The average duration of the complaint was 45 weeks. Seventy-one percent of patients had peripheral vestibular system dysfunction with benign paroxysmal positional vertigo (BPPV) being the cause of vertigo in one-third of these patients. Visual system disturbances were the primary diagnosis in 1% and proprioceptive disorders the primary diagnosis in 7%. Twenty-two percent were found to have metabolic or structural lesions of the brainstem. Psychiatric causes of dizziness were rare (43). In a study of 50 consecutive patients aged >60, symptoms of lightheadedness and syncope were associated with a cardiovascular etiology of dizziness, whereas the symptom of vertigo was associated with peripheral vestibular disorders (44). Most patients in this study complained of their presenting symptoms for >1 year, suggesting that diagnosis and treatment of many of these types of complaints is ineffective. (The average duration was stated to be 45 weeks. That makes it unlikely that ‘‘most’’ patients had their symptoms for more than a year—that is what was cited in the study.) Among the most common peripheral vestibular system pathologies is BPPV. In a cross-sectional study of patients complaining either of dizziness or balance disorders, unrecognized BPPV was found in 9% of patients. This diagnosis was associated with a history of falls, depression, and low activities of daily living scores (45). In a retrospective study of 1194 patients 70 years or older from a balance disorder clinic, 39% were found to have BPPV (46). This combination of findings suggests that all elderly patients complaining of dizziness, even when not complaining of the classic signs of BPPV, should be examined with a Dix-Hallpike maneuver.

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BPPV is easily treatable with either an Epley maneuver or a variety of home exercises. The outcomes of Epley maneuver versus more conservative treatment have not been specifically examined in the elderly. Developments in Vestibular Testing Changes in vestibular test results with age have been well documented. This would be expected given the histologic data cited above. Posturography has recently been used to investigate functional balance in the elderly. Wolfson et al. (47) examined 234 elderly subjects (average age 76) and compared them to 34 young control subjects. There was a significantly poorer in response to conflicting balance information when comparing the elderly and the young individuals (47). Cohen et al. (48) specifically evaluated four age cohorts (<44, 45–69, 70–79, and 80–89); they found a continual age-related decline in sensory organization test scores that persisted into the ninth decade of life. These changes were not, however, directly associated with decrease in independence (48). Baloh et al. (49) prospectively examined 72 subjects (age 79– 91) with normal neurological examinations and followed repeated posturography examinations on a yearly basis. They found that there was a significant increase in sway velocity and degree of sway with dynamic stimulation of balance over time. A standardized movement in the visual surround appears to increase sway and perturb balance to a greater degree in elderly subjects (50). Specific testing is needed to diagnose neurovestibular causes of dizziness and to determine fall risk. Treatment of Age-Related Balance Problems Few studies have rigorously looked at methodologies to improve balance in the elderly. Generally, balance disorders have been treated with physical therapy. The effect of Tai Chi was recently evaluated using posturography. Twenty-five test subjects who practiced Tai Chi were compared to 14 controls and were found to have statistically better outcomes on dynamic posturography (51). No long-term studies have correlated diagnosed peripheral vestibular system disease with treatment modality and outcome. There are also no prospective studies evaluating the pharmacologic treatment of vertigo. Summary Balance disorders are common and have a complex etiology. Even though balance disorders in the elderly can be related to a plethora of causes, histologic and epidemiological data suggest that a significant portion of balance disorders can be ascribed to disorders of the peripheral auditory system. No long-term prospective studies have combined the improvements in diagnostic techniques seen in the last 10 years with patient-based outcome measures. There are also little to no data available on the correlation between histological patterns of degeneration and changes in vestibular testing.

THE NOSE AND SINUSES Smell There has been little recent specific study of sinonasal disease in the elderly. Olfactory sensitivity has been found to decline with age. This may potentially be related

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to the degeneration of both peripheral and central olfactory pathways (52,53). One recent study has examined the smell sensitivity in normal elderly and patients with Alzheimer’s disease (54). The 80 patients with Alzheimer’s disease who were examined had awareness of olfactory sensitivity that was similar to that of chronic sinusitis patients (nine-fold less than normal elderly). Interestingly, 74% of the Alzheimer’s disease patients and 77% of the normal elderly who were found to have abnormal smell sensitivity rated themselves as having normal smell sensitivity on a questionnaire.

Sinusitis/Nasal Discharge A common symptom among elderly patients is postnasal drip that may be constant or induced by food (gustatory rhinitis). It is thought that this may be due to the loss of autonomic control, but it is more commonly a result of dehydration from reduced fluid intake or a side effect of medications (55). The allergy literature to some degree has addressed changes that occur during aging. Few studies have specifically examined the incidence or prevalence of sinusitis in the elderly. Knutson and Slavin (56) noted that sinusitis is common in the elderly and may have more subtle presenting signs. When sinusitis is properly treated, the management of asthma can be improved (57). The effect of sinus surgery for chronic sinusitis has been examined. In a study of 1112 patients who underwent endoscopic sinus surgery, patients aged older than 65 made up 15% of the patient population. This group had a higher incidence of minor complications, but final outcomes were similar to the other age groups (57).

TONGUE / ORAL CAVITY AND SWALLOWING Swallowing Normal Aging In a study of normal volunteers (n ¼ 80) divided into four age cohorts, liquid and semisolid swallows were studied with manometry and videofluoroscopy. Total swallowing time and time to initiation of oropharyngeal swallowing was prolonged in the advanced age. Upper esophageal sphincter pressure, peak pressure, and rate of bolus propagation did not appear to be affected by age greater than 65 (58). Other studies at least partially contradict these data, showing an overall slowing of pharyngeal swallowing time but also confirming an impairment in the opening of the upper esophageal sphincter (59). In a study of 53 asymptomatic individuals with an average age of 75, the repetitive oral suction test was applied to noninvasively evaluate swallowing function. Significant abnormalities in peak suction pressure, frequency of multiple swallows after one ingestion, frequency of polyphasic laryngeal movements, frequency of inspiration after swallowing, and frequency of coughing during or after swallowing were demonstrated. This suggested that a normal (non-dysphagic) population of patients has an increased incidence of a variety of physiologic abnormalities in the swallowing process and that concomitant disease may be more likely to result in pathologic dysphagia in the elderly who develop additional neurologic diseases (60–62). A series of elderly volunteers without symptoms of dysphagia were examined by endoscopy and fluoroscopy. Elderly subjects were found to

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require a much larger pharyngeal bolus to initiate swallowing (63). More recent studies have shown that with age there is an increase in pharyngeal swallow delay, a decrease in the duration of swallow, decreased cricopharyngeal opening, and decreased peristaltic amplitude and velocity (63,64). No studies have linked these changes or targeted treatment of these changes to improved nutritional status and quality of life in the elderly. Despite swallowing pressure remaining constant across aging, the reserve capacity of pressure generation within the oral cavity is reduced. It was argued that concomitant illness might thus put elderly patients more at risk of dysphagia due to reduced reserves (65). Normal aging does not appear to result in problems with the coordination of swallowing and protective deglutitive vocal cord closure (66). However, pressure sensitivity in the supraglottis of normal aging volunteers appears to decrease with age (67). This loss of sensory function may contribute both to dysphagia and aspiration in the elderly. More recent studies have looked at healthy individuals between the ages of 80 and 94. Again, increases in pharyngeal delay as well as a decrease in muscular reserve were demonstrated. The authors concluded that there may be a role for exercise to improve reserve and possibly prevent later swallowing disorders (68). Within the population aged greater than 65, 10% to 30% are estimated to have dysphagia, although this number is largely unsubstantiated (69). Some studies are beginning to address components of the swallowing system in a prospective fashion, allowing the establishment of measurement norms. Using four age cohorts (<50, 51–70, 71–85, and >85) with a sample of 30 in each, Jego et al. (70) studied by electromyogram (EMG) the activity of the mylohyoid muscle during swallowing. There were no significant differences among the groups. Rehabilitation of swallowing after head and neck cancer is vital for restoring the patient to a satisfactory functional state. Xerostomia may significantly contribute to dysphagia and is common among the elderly. The incidence of this problem is estimated to be as high as one of five elderly noninstitutionalized adults. A study of 67 randomly selected elderly subjects (institutionalized and noninstitutionalized) showed that there was a statistically significant (p < 0.001) association between xerostomia (measured by sialometry and questionnaire) and inadequacy of nutritional intake (71). There is evidence in the literature that there is a normal degeneration in salivary gland function with age (72). However, several studies based on actual measurements of salivary flow suggest that despite this histologic evidence of loss of salivary acinar structure, flow rates do not decrease with age. Fisher and Ship (73) examined unstimulated and stimulated salivary flow rates in healthy (non-xerostomia) subjects aged 20 to 40 and 60 to 80 years old. No differences in unstimulated or stimulated parotid flow rates were seen between the two groups (73). Dehydration and recovery from dehydration does not have an age-related differential effect on salivary flow (74). The largest study completed examined 1493 subjects between ages 5 and 88 using the whole saliva test rather than just isolating parotid salivary flows. Resting salivary flow was demonstrated to decline in an age-related fashion, thus contradicting studies based on parotid salivary flow alone (75). Besides functioning in lubrication during mastication, saliva also produces substances that are important for protecting the mucosal and dental surfaces of the mouth from infection. A recent study of 45 non-hospitalized dentate elderly (aged 79–89) and 22 non-elderly (aged 21–51) demonstrated that there is an age-related decline in secretory leukocyte protease inhibitor and lysozyme (antimicrobial proteins found in saliva). Overall, protein levels and electrolytes in the saliva have

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been shown to not change with age (76). Astor et al. (77) observed that medication (particularly anticholinergics and antipsychotics) and systemic illness are probably the most common causes of xerostomia. In an analysis of 100 consecutive patients aged >60 presenting at a xerostomia clinic, 60% were found to have salivary gland hypofunction. Of these, two-thirds were found to suffer from Sjorgen’s syndrome (78).

Summary There are clear age-related changes in the physiology of swallowing. Yet, for the most part, this does not appear to result in clinically significant dysphagia.

LARYNX Voice Many studies have looked at the effect of aging on voice quality. A basic description of age-related voice changes include an alteration in voice pitch and increased variability in pitch. Fundamental frequency of the voice increased with age for males and decreased with age for females. Estimated subglottic pressure was increased with increasing age. Overall, for unclear reasons, women were found to have less agerelated degeneration effects (79). In a longitudinal study of 20 patients, voice onset time and spectral features of the voice over 30 years were compared. The men tested showed a decrease in output in the 2–4 kHz part of the vocal spectrum. The voice onset time also became prolonged with age (80). The histological changes in aging vocal cords have been studied. There is an increase in fatty degeneration of the laryngeal muscles and a decrease in fiber density and elastin fibers in the vocal folds (81). Increasing ossification of the larynx with age alters the elastic and biomechanical properties of the insertion points of the vocal cords. There is a concomitant loss of sulfated glycosaminoglycans in the vocal ligament tendon, resulting in stiffening of the insertion zones (82). Several studies suggest that it is important to rule out a variety of disorders prior to making the diagnosis of ‘‘presbylarynges.’’ Woo et al. (83) retrospectively reviewed 151 patients over the age of 60 presenting for evaluation at a voice clinic complaining of dysphonia. Only six patients were found to fit the diagnosis of presbylarynges with vocal fold bowing and breathiness. The remaining patients were found to have voice changes related to central nervous system dysfunction (i.e., stroke, Parkinson’s disease), benign vocal cord lesions, inflammatory disorders, neoplasia, or vocal cord paralysis. In contrast, Hagen et al. (84) retrospectively reviewed 47 patients over the age of 60 presenting with dysphonia. This study found that up to 30% of these patients could be diagnosed with presbylarynges. Treatment for this disorder was speech therapy, with phonosurgery being reserved for failures of speech therapy. Tucker reported the results of vocal fold medialization using a modification of the Isshiki technique in six patients with the diagnosis of presbylarynges. The report claims that there are significant short-term benefits in terms of voice improvement; however, progressive relaxation of laryngeal tissues makes the long-term results of this procedure unsatisfactory (85). Currently, no studies using more current concepts of laryngeal framework surgery have focused on the elderly voice.

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Malignancies Complications After Laryngectomy In a retrospective study of 414 patients who underwent total laryngectomy, age greater than 65 or even age greater than 80 was not a variable contributing to medical or surgical complications (86). Pera et al. (87), as well as other retrospective studies, have confirmed these findings. In a study of 371 patients, Huygen et al. (88) also showed no increase in complications with age; however, age >70 was significantly predictive of death within three years after treatment. Patients older than 70 also have a higher incidence of developing a second primary after surgical or radiotherapeutic treatment of laryngeal cancer (89). Laryngectomy, though safe in the elderly, may have different long-term consequences. In a study of 58 patients post laryngectomy, a decrease in long-term expiratory function was found. This was more pronounced in patients over 65, even when age-related decrease in pulmonary function was considered. Treatment with bronchodilators reversed this trend (90). Head and Neck Cancer The effect of aging on head and neck cancer has been assessed in a number of retrospective studies. In these studies, the most common complications surveyed were mortality, myocardial infarction, pneumonia, pulmonary embolus, wound infection, post-op urinary tract infection, and postoperative bleeding. Change in mental status, percentage of patients who were converted from an independent to an institutionalized state, and incidence of decubitus ulcers were generally not examined. Management of cancers of the oral cavity is challenging from both an oncologic and a rehabilitative standpoint. A recent retrospective study in the British Journal of Cancer examined prognostic factors in the treatment of squamous cell carcinoma of the tongue. In stage I and II cancers, age > 65 was found to be a significant negative prognostic factor for survival. Age did not influence the prognosis of stage III and IV cancers (91). In a series of 187 poor-prognosis patients (recurrence of tongue cancer after radiotherapy), age at tumor presentation was found to be significant in determining outcome in men. Older men (age range 50–80) were statistically more likely to have shorter survival than age-matched women or younger individuals (92). Barzan et al. (93) examined prognostic factors in 438 patients with a variety of head and neck malignancies. Patients were divided into three groups by age. Age was not correlated with outcome. In a comparative study of major head and neck surgery, in 115 patients older than 70, Kowalski et al. (94) demonstrated that there was no agerelated effect on surgical mortality. This was interpreted as an indication to treat elderly patients with standard oncologic protocols. McGuirt and Davis (95) found an increased incidence of complications in head and neck cancer patients aged over 80, but also found that this group had a similar prognosis for survival and function as younger patients. In a retrospective cohort study, age over 65 or over 80 was not found to be a risk factor for the development of distant metastasis after resection of stage III/IV head and neck cancer. Screening for distant metastasis in geriatric patients should therefore depend on disease-based risk factors rather than age (96,97). Janot et al. (98) prospectively compared the predictive effects of clinical and pathological information in 108 patients presenting with head and neck cancer. Multivariate analysis showed that age and nodal status were prognostic for survival. Age, tumor status, and histological differentiation were predictive of metastatic disease. In a retrospective study of 207 patients, Magnano et al. (99) found no effect of age

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on the incidence of metastatic disease. Age also does not appear to have an effect on the radiosensitivity of tumors (100). Recent attempts at curative radiotherapy for head and neck cancer have used an accelerated dosing schedule. Age >70 years as an independent variable was not found to have an increased incidence of radiotherapy complications such as mucositis and weight loss. In terms of the defects and physical disabilities induced by aggressive resection of head and neck cancer, age does not appear to play a role. The development of microvascular techniques for reconstruction of defects of the oral cavity, pharynx, and hypopharynx has significantly improved the rehabilitation of these patients. Bridger et al. (101) retrospectively studied 26 patients older than 70 years and 91 patients younger than 70 years and found no differences in the postoperative complication rates for these two groups. Distribution of the type of cancer resected and the size of the defect were similar in the two groups. Shestak et al. (102) studied 92 patients in 10-year cohorts of age ranging from 50 to 80 years who underwent microvascular free flap reconstruction and found no statistically significant difference in complication rates in the patients ranging from age 70 to 79. Some older studies suggested that poor nutritional status may have more of an adverse effect on surgical outcome in older than in young patients. A retrospective study also demonstrated a significant difference in evaluation and preoperative treatment of malnutrition in elderly versus younger patients. Elderly patients had a higher complication rate when stratified by nutritional status, and younger patients were more likely to receive pre- and perioperative nutrition treatment (103). Summary From retrospective studies there appears to be no contraindication to standard oncologic treatment for head and neck cancer. Prospective studies are needed to confirm this. There is also little information on surgical decision making with regard to age.

CONCLUSIONS Review of recent reports addressing various disorders encountered in elderly patients with otolaryngologic complaints demonstrates that some of these disorders behave differently in this population when compared with younger patient groups. Knowledge of these variations is necessary to optimize management of the geriatric patient. Opportunities exist for further investigation of the unique characteristics and response to therapy in this age group. Otolaryngologists who treat elderly patients are encouraged to actively seek and critically assess reports of disease characteristics and management in this population.

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101. Bridger AG, O’Brien CJ, Lee KK. Advanced patient age should not preclude the use of free-flap reconstruction for head and neck cancer. Am J Surg 1994; 168:425–428. 102. Shestak KC, Jones NF, Wu W, Johnson JT, Myers EN. Effect of advanced age and medical disease on the outcome of microvascular reconstruction for head and neck defects. Head Neck 1992; 28:14–18. 103. Lin BS, Robinson DS, Klimas NG. Effects of age and nutritional status on surgical outcomes in head and neck cancer. Ann Surg 1988; 207:267–273.

4 Cerumen Impaction Cyrus Torchinsky and Terence M. Davidson Division of Otolaryngology—Head and Neck Surgery, VA San Diego Healthcare System, and Department of Surgery, UCSD Medical Center, San Diego, California, U.S.A.

INTRODUCTION Cerumen management is an integral and banal part of virtually every otolaryngologist’s practice. While the management of earwax often provokes ribbing from our more ‘‘macho’’ surgical colleagues, it remains an important quality-of-life issue for our patients. Perhaps nowhere else is this more true than in the elderly population where impacted cerumen is a common problem that often leads to decreased functionality and cognition. This chapter will be a compendium to assist practicing head and neck surgeons in managing cerumen in elderly patients and make suggestions for how to educate referring primary care physicians on how to manage cerumen in their office and when to refer for head and neck evaluation and assistance. To achieve this end, we will examine the anatomy and physiology of the external auditory canal as they relate to cerumen. This will include the recent biochemical efforts to determine the exact composition of cerumen so that a better ceruminolytic agent might be developed. Important in this discussion is the pathophysiology of cerumen impaction. The literature and current epidemiologic evidence examining the prevalence and impact of cerumen impaction in the elderly will be discussed. Then, after summarizing the limited clinical data on cerumen management, we will suggest our own practice algorithm for cerumen impaction. As a note, there is a semantic difference between cerumen, the product of specialized secretory glands and earwax, an amalgam of these secretions, and various other substances contained in the canal. Unless specified, we use these terms synonymously. After all, the offending substance we remove from our patients’ ears is earwax.

ANATOMY AND PHYSIOLOGY OF CERUMEN The external auditory canal is an approximately 2.5 cm long canal when measured from the external meatus to the tympanic membrane (TM). The lateral, cartilaginous part of the canal measures 8 mm. In the thick subcutaneous tissue in the outer or lateral third of the cartilaginous canal lie the numerous ceruminous and sebaceous 43

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glands that secrete what appears in the auditory canal as earwax (Fig. 1). Ceruminous glands are modified apocrine glands that have a coiled tubular structure resembling both sweat glands and secretory tissue in the breast (1). They lie deep to sebaceous glands, and in fact their ducts form a confluence with those emerging from the acini of sebaceous glands to empty adjacent to the hair follicles. Like all apocrine glands, the histology of ceruminous glands changes when glandular products are being secreted (2). While actively secreting cerumen, secretory cells have a columnar appearance. This changes to a cuboidal appearance when the glands are quiescent. Myoepithelial cells line the outer surface of the gland, aiding the propulsion of glandular products to the lumen of the external auditory canal (EAC). Earwax is a mixture of the products of ceruminous and sebaceous glands, large sheets of desquamated keratinocytes, shed hair, and any other substances that may have made their way into the external auditory canal (i.e., personal hygiene products, dirt, or, commonly in our Southern California practice, sand). There is a wide range of colors for cerumen. Some earwax is dark brown or black while some is a goldenyellow to light brown hue. It is believed that all cerumen begins golden in color and the combination of dehydration, oxidation, and bacterial activity is what leads to the darker colors (3). While the exact source of the pigmentation is unknown, recent efforts at determining the chemical composition of cerumen by Burkhart et al. may have yielded an answer. Not all cerumen is alike. Otolaryngologists working in communities with large Asian populations have long noted that these patients’ wax differs from that of Caucasians. Asians have dry, also known as ‘‘rice bran,’’ wax that is brittle, ash-like, and flaky appearing with a light grey to brownish grey color. This is opposed to the wet or sticky, gold to golden brown cerumen that is present in 98% of Caucasians

Figure 1 Low power (10X) paraffin-embedded section of an impacted cerumen plug demonstrating sheets of desquamated keratinocytes (center of figure).

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and those of African and Latin descent (4). Rice bran wax also distinguishes itself by being odorless and less copious than wet cerumen. Asians produce significantly less cerumen than whites, Africans, or Latinos. Some investigators feel that this is the result of fewer ceruminous glands in the canal (5). E. Matsunga in his 1962 paper ‘‘The Dimorphism in Human Normal Cerumen’’ described a simple Mendelian inheritance for cerumen phenotype. The exact locus of this gene was recently mapped by Tomita et al. (6) to the short arm of chromosome 16. This apparently inconsequential dichotomy of cerumen has yielded surprising avenues of research. Petrakis et al. (7) used cerumen phenotype to trace the migration across the Bering strait of native American peoples. This study was followed by work tracking the frequency and inheritance of cerumen phenotype in various tribes and led to the observation that the type of earwax is associated with breast secretions. Individuals with dry cerumen tend to produce fewer breast secretions. Given this correlation and the morphologic similarities between breast and ear tissue, Petrakis (8) extended this concept to the breast cancer finding that dry-type cerumen is associated with a reduced risk of this disease. Wet and dry are not the only two subtypes of cerumen. Hawke (3) has further carried this classification to distinguish between soft and hard cerumen. Hard cerumen is more often found in adults and is characterized as having a dry, desiccated consistency while soft cerumen is wet and sticky and is typified by what we see in the pediatric population. Histologically, soft cerumen has small sheets of keratin squames, shed cells also known as corneocytes from the stratum corneum in external canal skin, while hard cerumen has larger, more tightly packed sheets of desquamated keratinocytes. Corneocytes are expanded in soft cerumen, and not in hard cerumen. That earwax provides a protective function for the external auditory canal is undisputed. How it does this is a matter of some debate. It is certain that cerumen plays a role in lubricating and cleaning the external auditory canal. The chemical constituents of earwax are primarily hydrophobic compounds that help keep the otherwise damp, dark, and warm culture milieu [called the ‘‘greenhouse of the human body’’ by some (9)] of the external ear free of water. Cerumen is also very effective at trapping debris such as dirt, dust, shed hairs, and desquamated skin cells. The egress of this material is facilitated by the pattern of epithelial migration in the ear from the tympanic membrane outward. Jaw motion during mastication and speech further helps drive cerumen out of the canal. Cerumen has long been felt to act as protection against insects in the canal, although it is very difficult to scientifically verify this function (1). Whether or not cerumen has intrinsic antimicrobial activity is controversial. Some evidence suggests that this is the case. Cerumen has a pH ranging from four to five and has both bacterio- and fungostatic properties, and it contains such bacteriocidal materials such as lysozyme, IgA, and fatty acids (10). However, in vitro work has been inconsistent. Stone and Fulghum (11) found that populations of some bacteria were reduced 17% to 99% by a 3% suspension of wet cerumen. They tested seven species of bacteria including Staphylococcus aureus, Staphylococcus epidermis, Propionibacterium acnes, Corynebacterium spp., Eschericia coli, and Serratia marcescens. Their results implied that more pathogenic species were more susceptible to the effects of cerumen as were bacteria in the logarithmic rather than stationary phase of growth. No difference was found between the wet and dry types of cerumen. Similar findings were also reported by Chai and Chai (12), who suspended dry cerumen in a 3% glycerol–sodium bicarbonate buffer, finding up to a 99% reduction in growth for

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H. flu, E. coli, and Serratia, while growth rates were reduced by 30% to 80% for two Pseudomonas, one Streptococcus, and two Staphylococcus isolates. However, these results have been difficult to replicate. Campos et al. (13) found that cerumen suspensions more frequently increased rather than inhibited growth. A paper by Pata (14) showed reduction only in S. epidermis in cerumen from normal subjects. Intriguingly, the cerumen of patients with chronic otitis externa statistically inhibited growth of a pathogen, E. coli. Regardless of the end results of these studies, one thing that is clear is that at the very least cerumen acts a mechanical barrier to bacterial invasion of the very thin epidermis of the external auditory canal. Underproducers of cerumen are indeed predisposed to otitis externa, and the time-tested protocol of dry ear precautions for external otitis also gives hint that the drying properties provided by lipophilic compounds are important in preventing bacterial overgrowth of the external auditory canal. The regulation of cerumen production has been well studied, albeit some years ago. Adrenergic agonists, smooth muscle stimulants, and the emotional states of anxiety and fear in addition to resulting in increased production of apocrine sweat result in increased cerumen. Also, the mechanical actions of cleaning or rubbing the canal wall have a milking action on the ceruminous glands, helping to extrude their contents. Cerumen secretion can also be stimulated by vigorous chewing (5). In addition to the amount of cerumen extruded into the canal, the quality of this secretion can change. Measured over an entire population, cerumen can change its chemical composition over the course of the seasons. The triglyceride content of earwax decreases significantly in the transition from winter to summer months (15). This is felt to be a consequence of changes in diet from these times. The definitive studies of the biochemical composition of cerumen are the recent reports of Burkhart et al. (16,17). This group determined the constituent elements of wet hard cerumen in patients with obstructed ears. Their end goal is to use this information to discover a better ceruminolytic agent. Their first study determined the primary amino acid and simple sugar content of earwax. They found the major amino acid components to be glycine, glutamic acid, and serine. The most abundant carbohydrates in order of abundance were galactosamine, galactose, glucose, glucosamine, mannose, and fructose (16). This work was expanded one year later when the investigators used flash pyrolysis-gas chromatography/mass spectrometry to further elucidate the chemical composition of cerumen (17). The cerumen was first fractionated in a solution of deoxycholate, sodium chloride, and sodium phosphate. Then, the supernatant containing pure chemical constituents (i.e., the secretions of the ceruminous and sebaceous glands) and residue composed of squamous and proteinaceous debris were analyzed separately. This technique identified 152 principal compounds. These included aliphatic compounds, aromatics, nitrogen compounds, and possibly diterpenoids. This latter family of chemicals is quite interesting because among the diterpenoids are retinal and carotenoids. These compounds are abundant in plants and are major pigments in both fall foliage and carrots. The peak possibly representing diterpenoids was only found in the supernatant, indicating that Burkhart et al. (16,17) may have discovered the heretofore unknown pigment in earwax. The other peaks in their sample represented abundant squalene compounds and steroids. In addition to discovering the complexities of cerumen that yield its various physical properties [including some little known ones. For example, earwax was used as a binding agent for dyes in illuminated medieval manuscripts (18)], this study also yielded a potentially novel class of ceruminolytics: bile acids. Burkhart reported that primary and secondary bile acids in vitro were remarkably efficacious in dissolving

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the cerumen plug, perhaps due to the ability of the bile acids to form micelles with the cholesterol in cerumen. Although they suggest that they are developing clinical application of bile acids, no reports have emerged as of the time of this writing.

PATHOPHYSIOLOGY Subjective complaints from patients with cerumen impaction include itching, pain, hearing loss, tinnitus, vertigo, and chronic otitis externa. Other reported symptoms include chronic cough, perhaps mediated through the auricular branch of the vagus (Arnold’s nerve) that supplies the skin of the posterior and inferior wall of the canal (19). Hearing loss has been quantified by pure tone audiometric testing in patients with cerumen impaction. These audiograms demonstrate gradually decreasing highfrequency threshold sensitivity as percent of canal occlusion increases (20). With an 80% to 90% occlusion, hearing loss only occurred at frequencies greater than 1000 Hz with threshold decreases from 15 dB to 20 dB. Increasing the occlusion to 95% resulted in an extra 5–10 dB of threshold loss in higher frequencies, but no changes in frequencies less than 1000 Hz. Only with complete blockage of the canal do we see hearing loss in the lower frequencies with an average pure tone loss of 40 dB across the spectrum. This explains the commonly encountered clinical phenomenon whereby patients do not complain of hearing loss until their canals are nearly or completely occluded. While there is high frequency loss prior to 100% occlusion of the EAC, patients do not perceive their deficit until impaction becomes severe enough to impact the frequencies used in speech. In their review, Roeser and Ballachanda make the point that any deficits from cerumen impaction may in fact be compounded by pre-existing sensorineural loss. This is especially true in elderly patients who have a high prevalence of presbycusis (9). Another, perhaps often missed or misdiagnosed, consequence of cerumen impaction is decreased mental functioning. The association between cognitive impairment and hearing loss is inconsistently described. Peters et al. (21) found that hearing impaired individuals had a steeper decrease in their mental status scores over time compared with normal hearing individuals. Moreover, at least one study, Mulrow et al. (22), found that improvement of hearing was associated with improvement on the Folstein mini mental status exam, the Yesavage geriatric depression scale, and subjective surveys regarding emotional function and communication. Moore et al. studied how cleaning cerumen impaction influenced performance on the Folstein mini mental status exam (MMSE). In their cohort of 29 patients with cerumen impaction, this group found an average improvement of one point on the MMSE (23). Then, one must also consider the well-being of the elderly individual. A study examining quality of life and hearing impairment had 63% of respondents reporting severe emotional and social dysfunction as a result of their hearing loss. People with hearing loss also fare poorly on, and had ‘‘neurotic’’ or ‘‘borderline’’ profiles on, an anxiety questionnaire (24). The isolation caused by hearing impairment is difficult to quantify, but well understood by anyone regularly treating hearing impairment in a clinical practice. Whether cerumen impaction is a disorder of overproduction or failure of migration is not certain. There is histological evidence that individuals with cerumen impaction have more distended ceruminous glands relative to nonimpacted controls (2). However, this distension could as easily be the result of obstruction at the exit point for cerumen as a symptom of overactivity of ceruminous glands.

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The migration hypothesis as elucidated in a series of papers by Hawke’s group is the more compelling. Robinson of Hawke’s group examined impacted cerumen from 28 patients and found the combination of wet and hard cerumen to be the most associated with impaction (25). Robinson noted that these cerumen plugs contained more large sheets of keratin squames in them compared to earwax from nonimpacted ears. Clinically, this type of cerumen plug often has a pearly white color and can lead to pressure and pain within the EAC, as the dead cells have a tendency to absorb water and expand. The presence of these sheets of keratinocytes could be explained by a failure of the inward to outward migration of epithelium within the external canal. This failure of migration is hypothesized to be a deficit in a protein that cleaves cell–cell connections allowing corneocytes (keratinocytes in the stratum corneum) to separate from each other and cells in the stratum lucidum below. This to date undiscovered protein has been named KADS (keratinocyte attachment destroying substance), and it may turn out to be a crucial component of the ear’s self-cleaning mechanism. Without detachment, epithelial migration out of the canal is disrupted and the accumulating keratin ball serves to trap dirt, cerumen, and hair eventually forming a plug. To date, the only enzyme known to be involved in epithelial cell desquamation is steroid sulfatase. The target of steroid sulfatase is cholesterol sulfate, a known intracellular cement of sorts. In tissue from normal ear canals compared to those from patients with impacted cerumen, greater steroid sulfatase activity is seen in the deeper layers of the epithelium, supporting the hypothesis that this protein is involved with desquamation (dermatologists are well aware of disorders in keratinocyte detachment as they often treat patients with X-linked icthyosis). The desquamation hypothesis is also clinically relevant in the quest for the perfect ceruminolytic agent. In addition to addressing the chemical constituents of earwax, the ideal ceruminoltyic will be capable of disrupting the integrity of the keratin sheets in the ear canal (Fig. 1). This agent must be able to lyse the corneocytes’ cell membrane, allowing them to expand and separate from each other and adjacent cells in the stratum lucidum of the epidermis (3). The changes of normal aging also lead to a predisposition for cerumen impaction. As we age, the coarsening and lengthening of the hair within the external auditory canal acts as a physical impediment to self-cleaning of the ear. This is especially true in men. Additionally, there is a general decrease in the activity of cerumen glands in aging. This makes earwax drier and more difficult to clear from the canal. Another change of aging that can lead to cerumen impaction is the development of external ear canal exostoses. Finally, as the elderly are more frequent users of hearing aids, this cause of cerumen impaction must not be overlooked (26).

INCIDENCE The geriatric population has the highest incidence of cerumen impaction of any age group with the exception of the mentally retarded and residents of institutions. There have been three studies by two investigators examining the prevalence of pathologic quantities of cerumen in the elderly. Mahoney examined the problem twice, first in 1987 and then again in 1993. In the first study, Mahoney (27) found that 34% of 133 elderly subjects had impacted cerumen and another 23% had ‘‘moderate to large’’ amounts of cerumen in their EACs. This matched up well with the follow-up study showing 25% to 42% of ears in nursing home residents aged 62–100 had ‘‘moderate to large’’ amounts of impacted cerumen (28). Perhaps the most concerning fact reported

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by Mahoney was that in spite of the identification of excessive cerumen, most ears were either inadequately treated or untreated. This was attributed to the unwillingness of physicians to perform ear irrigations and lack of training/knowledge in the nursing staff on the use of ceruminolytic agents, ear irrigation, or otoscopy. Janken and Lewis-Cullinan reported similar results, with 35% of 226 individuals 65 or over having impacted cerumen. Nineteen percent of this cohort had bilateral impaction (29).

TREATMENT There are many safe and effective treatments for impacted cerumen. The challenge to the otolaryngologist is to determine which treatment to use with which patient. Before undertaking cerumen removal, a complete history should be taken. Has the patient had a history of this problem? Are there any signs suggesting tympanic membrane perforation? Were there complications of prior cerumen removals such as dizziness, bradycardia, etc.? This information is important because there have been reported cases of cardiac arrest arising from manipulation of the EAC, thought to be a response mediated by Arnold’s nerve (30). Also one must know if this is an only hearing ear, for if so irrigation is contraindicated and the cerumen must be cleaned by direct visualization. Before inserting an ear speculum, the external auditory meatus and canal should be inspected for signs of bleeding, erythema, swelling, or crusting to rule out a concomitant infection. The canal is best examined by pulling the pinna of the ear superiorly and posteriorly. If the speculum is inserted superiorly and anteriorly the drum should be visualized. In cases of impaction, one will see a plug of wax ranging from black to dark brown to white in color. The treatment should proceed as follows. The majority of cases of cerumen impaction can be cleared by irrigation of the canal. Most otolaryngologic practices have a compressed air irrigation setup on hand, but a 20 cc syringe with a 14-gauge Angiocath can be used in lieu of more elaborate equipment. Commercial irrigators are also available, most often used in pediatrics. Typically, ears are irrigated with water, but some choose to add alcohol, hydrogen peroxide, or Burrow’s solution. No one solution has a demonstrated clinical benefit over another solution. The irrigant must be warmed to body temperature to prevent stimulation of the vestibular system. When introducing the irrigant, care is taken to direct the stream laterally in the EAC. This prevents injury to the tympanic membrane as well as allowing the jet of water to pass behind the cerumen plug facilitating its egress from the canal. It is judicious to follow any irrigation procedure with a few drops of an antibiotic ear solution (31). If irrigation fails, the next steps are the introduction of a ceruminolytic followed by removal under binocular otomicroscopy. Surprisingly, when one reviews all the literature involving the ideal ceruminolytic, no consensus arises to what is the best agent. Hydrogen peroxide, Burrow’s solution, and acetic acid have been studied and proven to be of some benefit over no treatment, but no solution has consistently outperformed warmed tap water (32). Mineral oil is also used by many. Pediatricians have long been using docusate sodium as a ceruminolytic, and it remains the agent of choice in a handful of academic otolaryngology departments (33). The key to the use of any ceruminolytic is that it be warmed before introduction to the canal. Some authors claim this is especially true for Colace.

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The ceruminolytic should be given five or so minutes to work on dissolving or loosening the plug before removal is attempted. How one proceeds from there is largely determined by what is seen in the canal. One preferred method is to use a five French otologic suction and roll the cerumen plug into a ball. (While suctioning the EAC be aware that many patients do not tolerate this procedure without discomfort. The noise is loud and it is possible to induce vertigo with this procedure, i.e., an air caloric stimulation.) The cerumen can in most instances be sucked out of the canal. If suction fails, with extreme care a Rosen hook may be introduced into the canal and either wedged behind or even into the cerumen plug to pull it out. Alternately, propitious use of a cerumen loop and cup forceps can be very effective. Any instrument used in the canal should have smoothed edges to avoid injury to canal skin. Given the high incidence of atelectatic drums in the elderly, extreme caution must be taken not to push the wax ball inwards. There are multiple documented cases of tympanic membrane perforation and even incostapedial dislocation that led to permanent sensorineural hearing loss (34). Caveats also include the thinned skin in the elderly. Even in young subjects there is no deep dermal tissue overlying the bony canal. With the thinning of skin that occurs with aging, interventions performed in the medial EAC can be extremely painful. It is also very easy to induce bleeding, which in addition to clouding the field of the microscope is of some distress to the patient and indicates a violation of EAC skin that may predispose to infection. If the wax plug is recalcitrant to the above interventions, it is best to stop, have the patient go home on a weeklong regimen of your preferred ceruminolytic, and then follow up for removal.

COMPLICATIONS OF CERUMEN REMOVAL Complications of cerumen removal can be protean and should be managed appropriately. As mentioned above, the already thin skin of the canal is further thinned by aging. Coupled with the fact that many elderly patients are on aspirin or other blood thinners, bleeding is a common complication. If bleeding arises, antibiotic otic drops can be administered. With a severely tender and edematous canal, oral antihistamines may be of some benefit to patient comfort and in fact speed healing. Tympanic membrane perforation is another common complication. Small perforations usually heal on their own, but this can take from weeks to months in a geriatric patient. Also, tympanic membrane perforation can be associated with injury to the ossicles or even worse, perilymph fistula. The latter condition is especially suspected when nystagmus, tinnitus, and hearing loss follow treatment. Tinnitus without nystagmus may occur in absence of injury to the ossicles or even without a TM perforation. However, this form of tinnitus is usually self-limiting. If one is suspicious of perilymph fistula or ossicular injury, prompt referral to audiology for a fistula test and measurement of stapedial reflexes is indicated. Virtually all of the major complications of cerumen removal: TM perforation, ossicular disruption, and perilymph fistula are amenable to surgery. However, diagnosis must be made rapidly. If a fistula is indeed present per a fistula test by an audiologist, the patient should follow four to five days of bed rest and avoid Valsalva maneuvers. Use of stool softeners is encouraged if constipation is an issue. Perilymph fistulas typically present with a sensorineural hearing loss of 30–40 dB. This can be hard to parse out if there was preexisting sensorineural hearing loss (SNHL) in the patient. A 30 dB air bone gap usually indicates injury to the conductive component of the ear caused by the trauma of irrigation or too forceful removal with instruments (31).

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Nausea and vomiting are common complications, which can be avoided by warming irrigation fluid and taking care to avoid suction in the context of a perforated tympanic membrane. Less common complications include cardiac arrest, visual changes, and otitis externa following cerumen removal. There has been a large rise in the use of ‘‘alternative’’ medicines and therapies in the last 10 years. One such remedy is the ear candle, a homeopathic treatment that involves placing a hollow candle into the EAC and lighting the opposite end. This ostensibly creates a vacuum that sucks cerumen out. After the treatment, a substance resembling wax is left in the candle stub. This treatment has been touted in the popular press as being safe, effective, and cheap. While the cost of ear candling is indeed approximately one-fifth that of removal in a doctor’s office, this practice is neither efficacious nor safe (35). None of the 20 candles tested generated negative pressure in a specially designed tympanometric setup. None of the impacted ears were cleared of cerumen, and in fact the investigators halted their study early due to injurious effects of the treatment. The wax-like substance in the candle stubs was examined with a mass spectrometer and found to have no earwax constituents, but it was rich in the alkanes that are commonly found in candle wax. A survey sent out by Seely’s group found that burns to the auricle or EAC were common as were complete or partial occlusions of the EAC with candle wax and there was one report of a tympanic membrane perforation. Thus, otolaryngologists must be aware of this practice, counsel against its use, and be prepared to treat complications.

OUR PRACTICE ALGORITHM Cerumen impaction in the elderly is a problem that should be primarily managed by primary care physicians. We suggest a simple algorithm that can be distributed to primary care physicians delineating management and referral guidelines (Fig. 2). Irrigation is the simplest and most readily available choice for impacted ears. Thus, this should be the first-line treatment using any of the base irrigants mentioned above (i.e., water warmed to body temperature with or without a combination of alcohol, hydrogen peroxide, vinegars, or Burrow’s solution). Contraindications to irrigation include patients with chronic ear disease, a known tympanic membrane perforation, or an only hearing ear. These patients should have their cerumen removed under the binocular microscope and thus should be automatic referrals. If the initial irrigation is not successful, a warmed ceruminolytic agent is instilled into the ear canal. After 15 minutes, the irrigation process is repeated. If the plug remains, the patient is sent home with a cerumen softener such as mineral oil. One week later the process is repeated. Failure to clear the plug necessitates referral to a specialist. When performing irrigations, physicians must know when to stop the procedure to avoid the aforementioned complications. To ensure that there is no reaccumulation of cerumen, patients can be instructed to use the otic preparation of Burrow’s solution: Domeboro otic drops, two drops into each ear two to three times a week after showering. Burrow’s solution, containing aluminum acetate, is an astringent which helps denature protein and by stimulating the EAC epithelium nurtures outwards epithelial migration. Many patients will only suffer clinically apparent cerumen impaction every two or so years. Primary care physicians can easily manage these patients when they have problems. For those patients desiring more definitive management or in whom cerumen impaction is a chronic problem, we have two treatments. The first is the

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Figure 2 University of California–San Diego cerumen impaction algorithm.

abovementioned Domeboro otic prophylaxis. Alternatively, patients can instill two to three drops of mineral oil into each ear canal weekly. This can be followed by irrigation with a small bulb syringe.

HISTORICAL PRECEDENT AND CONCLUSIONS There is nothing new under the sun, but there are lots of old things we don’t know. —Ambrose Bierce (1842–1914)

Ambrose Bierce’s take on the famous quote from Ecclesiastes was made all too real in the final stages of preparing this chapter. A review of a textbook published in 1902 revealed that the otolaryngologists of that time had both a thorough understanding of the mechanisms of cerumen impaction as well as treatments that have been lost in the intervening century. We found this so interesting that the section on cerumen management is reproduced below for those who wish to read it.

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Impacted Cerumen—Occlusion of the auditory canal by a mass of inspissated cerumen is the affection of the external ear which most frequently demands the attention of the aurist. Usually the patient is entirely unconscious of the presence of the mass until the canal is completely occluded by it. Then the hearing, which before had not been appreciably diminished, although the ceruminous plug may have all but filled the lumen of the canal, becomes at once greatly impaired, autophony manifests itself, and very frequently tinnitus makes its appearance to add to the discomfort and alarm of the patient. Although the mass develops very slowly, many months usually elapsing before it becomes inconveniently large, the symptoms just enumerated generally manifest themselves suddenly. The usual explanation of this is that water has run into the ear in washing or bathing, or in warm weather perspiration has found its way into it and has caused the plug, which previously had nearly filled the canal, to swell up sufficiently to make the occlusion complete. Occasionally it is a fluid which the individual has intentionally dropped into the ear or some manipulation on his part of the ceruminous mass, which brings about the sudden change. Exceptionally the same symptoms may result, accompanied, perhaps, by pain, from a smaller mass of wax (which left undisturbed might not have caused inconvenience for a long time) being dislodged and pushed down upon the drumhead by the efforts of the patient to remove it. Again, when the plug is very hard and occupies the outer portion of the meatus, it may, through the movements of the jaw, exert sufficient pressure upon the canal walls to cause pain, and perhaps inflammation, before it has become so large as to interfere with audition. The plug varies greatly in consistency and in solubility, and frequently contains innumerable short, pale hairs (from the walls of the canal). Very often it is in part made up of pieces of exfoliated epidermis, and exceptionally it has as a nucleus some small foreign body which has found its way into the ear, or an old scab left by a former otitis. More frequently than not both ears are involved, so both should invariably be examined. Among the rarer symptoms produced by the presence of impacted cerumen in the ear may be mentioned dizziness, reflex cough, perturbation of the mental faculties with inability to concentrate the mind in intellectual pursuits, disturbances of gait simulating those of locomotor ataxia (Risley), epileptiform convulsions, and, in a case reported by the writer (2), inability to swallow, accompanied by a feeling of oppression about the heart. The added danger which results in otitis media from a pre-existent occlusion of the meatus by impacted cerumen should also not be lost sight of. With good illumination it is usually a very easy matter to detect the presence of a ceruminous plug in the auditory canal. It is seen as a dark brown mass filling the lumen of the canal, with its outer surface situated usually at about the line of juncture of the osseous and membranous portions of the meatus. Touched with a probe, it may appear quite hard, or may be soft and easily indented. Generally the inner extremity of the mass reaches to, and rests upon, the tympanic membrane. The etiology of this affection has received considerable attention, and, while it cannot be claimed that it is as yet fully understood, there is a general agreement at least as to two points: in the first place, that, probably through reflex influence, the ceruminous glands are frequently abnormally active in the presence of chronic inflammatory affections of the nasopharynx; in the second place, that under such circumstances and often perhaps independently of such conditions, there is a disturbance of the normal outgrowth of the epidermis which covers the external

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surface of the drumhead and lines the walls of the meatus. This in health tends to transport the cerumen from the deeper portions of the canal to its external orifice, where it falls out or is removed in the ordinary daily ablutions. That catarrh of the nasopharynx is frequently present when there is a disposition to the formation of ceruminous plugs in the ears is a fact of daily observation, and there can be little doubt that it is an important factor in their causation. And the composition of many masses of impacted cerumen—made up, in great part, of layers of exfoliated epidermis, and sometimes enclosed in a thin pouch of epidermis which has been cast off entirely from the tympanic membrane and the walls of the meatus—would seem to show that under certain circumstances there is not only an arrest of the normal outgrowth of the epidermis, but an actual reversal in the direction of its growth, tending to a heaping up of epithelial de´bris in the deeper parts of the canal, as well as to an impaction of cerumen. Treatment—It would seem that as to the manner of dealing with so simple a condition there could be but little room for difference of opinion, still less for contention. Such, however, is far from being the case, for one very high authority tells us in his excellent treatise upon diseases of the ear that the syringe should rarely be used for the removal of cerumen, and that with the curette and the angular forceps one may accomplish in ten or fifteen minutes what cannot be done with the syringe in an hour’s time; while another excellent authority tells us in his book that in four or five years he has not met with a single instance in which by means of the syringe he has failed to remove impacted cerumen from the ear in one sitting of five minutes or less, and that as to the curetting method he feels that he cannot seriously argue the question. At the risk of seeming to be contentious himself, the writer cannot refrain from saying that this last expressed sentiment meets with his fullest endorsement. But still another very high authority, whose example in most things we are glad to follow, actually commends the introduction of a strong solution of caustic potash into the ear (of course with the exercise of extreme caution) in order to saponify quickly the ceruminous mass and so to facilitate its removal. As to this procedure, it may be remarked that in kindling, and especially in rekindling a fire, petroleum is a great saver of time; but, even so, it is not the part of wisdom to commend its general use in this way. The method of dealing with impacted cerumen which the writer has found most convenient, and which he has employed for many years, is as follows: In the great majority of cases the syringe is chiefly relied upon. When, however, the ceruminous mass proves obdurate and does not easily undergo disintegration, the angular probe or the instrument for the removal of foreign bodies represented in Figure 490 is brought into requisition and the mass is partly broken up or separated from its attachment to the canal wall. After this the syringing is resumed, and usually with much better effect. Bicarbonate of soda is invariably added to the warm water (105–110 F) with which the syringing is done, as it unquestionably facilitates the removal of the wax and certainly does no harm to the syringe, as has been suggested. The quantity used is never accurately determined, but is approximately half an ounce to a quart. The ear is inspected from time to time to make sure that there is still cerumen in it, and that the syringing is not being kept up unnecessarily. As the mass diminishes in size and there is a likelihood that the stream of water may impinge upon the drumhead, the force with which it is thrown into the ear is lessened. The exact direction in which the stream strikes the impacted mass is not thought to be of especial moment, and no apprehension

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is felt that this may result in the plug being driven by the force of the water more deeply into the meatus, as some have imagined. When both ears are affected, unless the mass first attacked comes out very readily, the syringing is alternated from one ear to the other, as this saves time and appreciably diminishes the amount of syringing required. The intermittent stream of a piston syringe is employed, and is thought to be more efficacious than the continuous stream of a fountain syringe. The hard-rubber, kidney-shaped basin commonly employed by aurists has been long since discarded, because it is concave where it should be convex, and so does not fit well into the hollow beneath the ear, and because, moreover, it is so long and shallow that a very slight movement on the part of the patient is likely to cause its contents to slop over upon the clothing. Instead of this, a china bowl (one made of hard rubber or metal might be better, because less fragile) of the shape represented in Figure 489 is used, and has been found much better adapted to the purpose, since it is free from both of the faults mentioned. It is always held by the patient, over whose shoulder a napkin is spread, rather than by an assistant, unless the patient be a young child. When inspection with the speculum and mirror shows that all of the cerumen has been removed, two or three syringefuls of plain warm water are gently thrown into the ear to wash out the previously used soda solution. The ear is then dried with a spill of soft linen and closed with a bit of absorbent cotton, which in cold weather the patient is advised to wear until bedtime. If the plug proves to be exceptionally refractory or time be pressing, the patient is told to report the next day, and in the meantime to drop into the ear several times a little warm sweet oil, or, if it be inconvenient for him to do this, the ear is filled with a saturated solution of soda, and after perhaps a half-hour’s wait the syringing is resumed. The cases in which the plug cannot be removed at one sitting are very exceptional, but the writer is compelled to admit that with the best skill he can command it is not unusual for him to spend many more than ‘‘five minutes’’ in accomplishing this result. It occasionally happens that upon inspecting the ear it can be seen that the mass of cerumen does not extend into the deeper parts of the canal. Under such circumstances, if it is found to be of its usual firmness, it is often possible with the traction instrument (Fig. 490) to draw out the whole mass at a single effort, and so to save both time and trouble. If, however, even in such a case, the cerumen proves to be of such consistency that it can be removed only bit by bit, it is better to resort to the syringe without further ado. The writer knows of no means by which the well-recognized disposition of impacted cerumen to recur after having been removed can be overcome, except in so far as the cure or amelioration of any accompanying inflammation of the nasopharynx tends to this result. In summary, practicing otolaryngologists must have an understanding of the basic science of cerumen. Earwax is produced in the outer third of the cartilaginous canal and is composed of glandular products, desquamated skin, and whatever manages to get into the canal. There are ethnic morphological differences between dry, Asian cerumen, and the wet cerumen found in all other races. Whether or not cerumen has true antibiotic properties has been difficult to verify in vitro, but at the very least it helps keep the canal dry and protects the fragile EAC skin. Sophisticated biochemical techniques have been applied to determine the exact composition of cerumen and aid in the development of better ceruminolytics.

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Earwax is cleared by the epithelial migration from the tympanic membrane to the auditory meatus as well as mechanical forces milking the canal during mastication. Recent work has rediscovered that cerumen impaction is more likely the result of a failure of the detachment of keratinocytes than overproduction. This disease process is likely genetic and exacerbated by the changes of aging. It is important that this easy to detect condition be diagnosed as the manifestations of disease are protean and treatments easy to administer. Treatment of cerumen impaction is straightforward and most cases can be managed with a combination of irrigation and ceruminolytics. All agents work reasonably well, but there is still room for improvement. Early 20th century head and neck physicians used potash, a compound made by percolating water through a mixture of wood ash and lime (36). Perhaps not so ironically, potash is a saponifying agent like the bile acids recently suggested to be the optimal ceruminolytic. Referral to otolaryngologists should be made for patients with TM perforations, chronic ear disease, impacted only hearing ears, and impacted cerumen not easily cleaned by the primary care physician. Cerumen impaction prophylaxis can be obtained with Domeboro otic drops to encourage migration out of the EAC or with weekly application of mineral oil with or without self-irrigation.

REFERENCES 1. Kelly KE, Mohs DC. The external auditory canal. Anatomy and physiology. Otolaryngol Clin North Am 1996; 29(5):725–739. 2. Mandour MA, El-Ghazzawi EF, Toppozada HH, Malaty HA. Histological and histochemical study of the activity of ceruminous glands in normal and excessive wax accumulation. J Laryngol Otol 1974; 88(11):1075–1085. 3. Hawke M. Update on cerumen and ceruminolytics. Ear Nose Throat J 2002; 81(8 suppl 1): 23–24. 4. Matsunaga E. The dimorphism in human normal cerumen. Ann Hum Genet 1962; 25: 273–286. 5. Perry ET. The human ear canal. Springfield, IL: Charles C. Thomas, 1957. 6. Tomita H, Yamada K, Ghadami M, et al. Mapping of the wet/dry earwax locus to the pericentromeric region of chromosome 16. Lancet 2002; 359(9322):2000–2002. 7. Petrakis NL, Molohon KT, Tepper DJ. Cerumen in American Indians: genetic implications of sticky and dry types. Science 1967; 158(805):1192–1193. 8. Petrakis NL. Cerumen phenotype and epithelial dysplasia in nipple aspirates of breast fluid. Am J Phys Anthropol 1983; 62(1):115–118. 9. Roeser RJ, Ballachanda BB. Physiology, pathophysiology, and anthropology/epidemiology of human earcanal secretions. J Am Acad Audiol 1997; 8(6):391–400. 10. Osborne JE, Baty JD. Do patients with otitis externa produce biochemically different cerumen?. Clin Otolaryngol 1990; 15(1):59–61. 11. Stone M, Fulghum RS. Bactericidal activity of wet cerumen. Ann Otol Rhinol Laryngol 1984; 93(2 Pt 1):183–186. 12. Chai TJ, Chai TC. Bactericidal activity of cerumen. Antimicrob Agents Chemother 1980; 18(4):638–641. 13. Campos A, Betancor L, Arias A, et al. Influence of human wet cerumen on the growth of common and pathogenic bacteria of the ear. J Laryngol Otol 2000; 114(12):925–929. 14. Pata YS, Ozturk C, Akbas Y, Gorur K, Unal M, Ozcan C. Has cerumen a protective role in recurrent external otitis? Am J Otolaryngol 2003; 24(4):209–212. 15. Cipriani C, Taborelli G, Gaddia G, Melagrana A, Rebora A. Production rate and composition of cerumen: influence of sex and season. Laryngoscope 1990; 100(3):275–76.

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16. Burkhart CN, Burkhart CG, Williams S, Andrews PC, Adappa V, Arbogast J. In pursuit of ceruminolytic agents: a study of earwax composition. Am J Otol 2000; 21(2):157–160. 17. Burkhart CN, Kruge MA, Burkhart CG, Black C. Cerumen composition by flash pyrolysis-gas chromatography/mass spectrometry. Otol Neurotol 2001; 22(6):715–722. 18. Petrakis NL. Earmarks of art history: cerumen and medieval art. Am J Otol 2000; 21(1):5–8. 19. Raman R. Impacted ear wax—a cause for unexplained cough? Arch Otolaryngol Head Neck Surg 1986; 112(6):679. 20. Chandler JR. Partial occlusion of the external auditory meatus: its effect upon air and bone conduction hearing acuity. Laryngoscope 1964; 74:22–54. 21. Peters CA, Potter JF, Scholer SG. Hearing impairment as a predictor of cognitive decline in dementia. J Am Geriatr Soc 1988; 36(11):981–986. 22. Mulrow CD, Aguilar C, Endicott JE, et al. Association between hearing impairment and the quality of life of elderly individuals. J Am Geriatr Soc 1990; 38(1):45–50. 23. Moore AM, Voytas J, Kowalski D, Maddens M. Cerumen, hearing, and cognition in the elderly. J Am Med Dir Assoc 2002; 3(3):136–139. 24. Mulrow CD, Aguilar C, Endicott JE, et al. Quality-of-life changes and hearing impairment. A randomized trial. Ann Intern Med 1990; 113(3):188–194. 25. Robinson AC, Hawke M, Naiberg J. Impacted cerumen: a disorder of keratinocyte separation in the superficial external ear canal? J Otolaryngol 1990; 19(2):86–90. 26. Naiberg JB, Robinson A, Kwok P, Hawke M. Swirls, wrinkles and the whole ball of wax (the source of keratin in cerumen). J Otolaryngol 1992; 21(2):142–148. 27. Mahoney DF. One simple solution to hearing impairment. Geriatr Nurs 1987; 8(5):242–245. 28. Mahoney DF. Cerumen impaction. Prevalence and detection in nursing homes. J Gerontol Nurs 1993; 19(4):23–30. 29. Lewis-Cullinan C, Janken JK. Effect of cerumen removal on the hearing ability of geriatric patients. J Adv Nurs 1990; 15(5):594–600. 30. Prasad KS. Cardiac depression on syringing the ear. A case report. J Laryngol Otol 1984; 98(10):1013. 31. Grossan M. Safe, effective techniques for cerumen removal. Geriatrics 2000; 55(1):80, 83–86. 32. Burton MJ, Doree CJ. Ear drops for the removal of ear wax. Cochrane Database Syst Rev 2003;(3):CD004400. 33. Chen DA, Caparosa RJ. A nonprescription cerumenolytic. Am J Otol 1991; 12(6):475–476. 34. Dinsdale RC, Roland PS, Manning SC, Meyerhoff WL. Catastrophic otologic injury from oral jet irrigation of the external auditory canal. Laryngoscope 1991; 101(1 Pt 1): 75–78. 35. Seely DR, Quigley SM, Langman AW. Ear candles—efficacy and safety. Laryngoscope 1996; 106(10):1226–1229. 36. Theobald S. Affections of the external ear. In: deScheintz GE, Randall BA, eds. An American Text-Book of Diseases of the Eye, Ear, Nose, and Throat. Philadelphia and London: WB Saunders and Co., 1901:699–703.

5 Amplification and the Geriatric Patient Catherine V. Palmer Communication Science and Disorders, University of Pittsburgh, and Audiology and Hearing Aids, Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, U.S.A.

Amanda Ortmann Communication Science and Disorders, University of Pittsburgh, and Department of Audiology and Speech Language Pathology, VA Pittsburgh Health Care System, Pittsburgh, Pennsylvania, U.S.A.

GENERAL DESCRIPTION OF THE CLINICAL PROBLEM Improvements in technology and medicine over the past 50 years have resulted in significant increases in life expectancy. Twelve percent of the U.S. population was over the age of 65 in 2000; by 2050 that figure will nearly double (1). Along with the shifts in the demographics, there also will be a shift in the health status, with an increase in the number and severity of chronic health conditions affecting the population (2). Hearing loss is the third most commonly reported chronic condition within the geriatric population (behind arthritis and hypertension) (2). According to the National Center on Deafness and Other Communication Disorders, 33% of the population over the age of 60 has hearing loss, increasing to 50% of those over 80 (3). Eight out of 10 residents of nursing homes suffer from hearing loss (4). Although hearing impairment is a prevalent problem among the elderly, only 20% of those affected actually use hearing aids (5). This is in remarkable contrast to visual impairment; 93% of the elderly population with visual problems wear glasses routinely (6). The sequellae of uncorrected hearing loss are very similar to those of visual impairment: decreased mobility, social isolation, and depression (7). Hearing aids and other assistive devices provide significant improvement of not only hearing function, but also of the overall quality of life of the elderly individual. WHY IS HEARING LOSS PARTICULARLY IMPORTANT IN THE GERIATRIC POPULATION? Currently, aging adults account for 43% of the hearing-impaired population (8). As the median age of Americans rise, an increase in the prevalence of hearing loss is to be expected. Health care professionals can assist with the needs of their 59

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elderly patients by understanding the nature, repercussions, and remediation of hearing disorders. Sensory impairments can limit the quality of life for both elderly individuals and their families. Uncorrected hearing loss leads to reduction of functional, psychological, and psychosocial well-being (9–11). Studies by Mulrow and colleagues (12) as well as Weinstein and Ventry (13) have found that even a mild to moderate hearing loss has a considerable effect on social and emotional handicaps (12,13). The elderly individuals judge the hearing handicap as being less severe than their spouse or partner (14). Thus, family members are often first to recognize the problem of the individual’s hearing loss and encourage the use of amplification. Telephone communication often becomes an important link to family and safety as mobility is reduced with aging, with a concomitant requirement for amplification. There is early evidence that uncorrected hearing loss may contribute to cognitive dysfunction (15). Uhlmann and colleagues (16) examined the relationship between hearing impairment and dementia and found that the prevalence of hearing impairment was higher in the demented adult sample than a comparable sample of adults free from dementia. Dementia and hearing loss interact to produce a more rapid cognitive decline in elderly individuals (17). Management of hearing loss in the demented population is problematic (18). Although viewed by health care professionals as being hard to test and manage, these patients may often benefit from evaluation and amplification (19).

BASIC SCIENCE Presbycusis disrupts both the peripheral and central portions of the auditory system (20). The peripheral loss of hair cells results in decreased audibility of the acoustic signal, particularly in the higher frequencies beyond 2000 Hz. The consonant properties of speech such as the ‘‘s,’’ ‘‘sh,’’ ‘‘f,’’ ‘‘t,’’ and ‘‘p’’ have acoustic energy within the range of 2000–8000 Hz and are therefore ‘‘dropped.’’ Vowel properties of speech are preserved; hence, aging adults typically state that they hear conversations, but cannot understand what is being said. The central component of presbycusis results in a degradation of the rapid processing of speech information, the ability to localize, and the ability to use binaural cues, and increases in severity with increasing age (21,22). Binaural cues are critical for separating speech from background noise. The elderly patient will often have difficulty understanding speech in the presence of background noise due to the central effects of aging, even in the presence of adequate amplification. An aural rehabilitation program should include not only amplification but also education regarding the use of various communication strategies, assistive listening devices (ALDs), and assertiveness.

HISTORY Figure 1 displays a typical audiogram and word recognition results of an elderly individual. Hearing function is similar in both ears with normal or near-normal lowfrequency hearing and decreased hearing sensitivity in the higher frequencies. This individual notes that this hearing loss has a negative effect on communication abilities and lifestyle. The audiogram suggests that the individual is a candidate for an aural

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Figure 1 Audiogram of a typical aging hearing loss.

rehabilitation program. However, more information (history) about the individual is required in order to select the proper amplification device and counseling strategies. Physical, psychosocial, or psychological changes associated with aging influence the auditory testing procedures and the aural rehabilitation design and outcome (10). Foremost among the physical changes that can interfere with the aural rehabilitation program are the individual’s overall general health, visual deficits, and dexterity. Both advanced age and concomitant disease(s) may influence the individual’s motivation and acceptance of the aural rehabilitation program. Manipulation of a hearing aid or other assistive listening device requires visual acuity and fine motor skills. Although not a contraindication, vision loss and decreased dexterity must be considered in the context of the aural rehabilitation program. Cerumen production is a challenging physical change that impacts aural rehabilitation, as it can lead to malfunctioning of hearing aids by blocking the receiver opening in certain instruments. Some elderly hearing aid users require routine cerumen removal. A major psychosocial change in aging adults that affects the aural rehabilitation program is the reduction of social activity. This reduction may or may not be the direct result of hearing loss. In order to be motivated to participate in a program designed to improve communication function, the aging individual must be involved in meaningful social interactions. Hearing-impaired adults experience some degree of social isolation and often require encouragement to seek increased social activities as part of their rehabilitation program. Psychological changes such as cognitive decline and depression also can influence the diagnosis and treatment of hearing impairment in the elderly population. Hearing loss has been associated with a sense of helplessness, passivity, and negativism (23). These negative emotions will influence the aging patient’s acceptance of the hearing aids. Aging individuals may need a longer timeframe to learn and retain new information (24).

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PHYSICAL EXAMINATION A case history is an integral component of every audiologic and/or hearing aid evaluation. It consists of specific questioning regarding hearing, tinnitus, balance function, situational performance, and general health. Otoscopy is performed with particular attention to ear texture and elasticity and checking for collapsing ear canal openings. These findings impact the choice of earphones for audiometric testing and later impact recommendations related to hearing aid selection and earmold material. The ear canal must be free from debris and the tympanic membrane visible and intact.

TESTING Screening The onset of presbycusis is subtle, and patients are frequently unaware of their hearing loss and do not seek evaluation of their hearing voluntarily. Routine screening of hearing loss by primary care physicians may be quite helpful in the detection of patients who would benefit from aural rehabilitation. Screening may be accomplished through a handheld device that functions as both an otoscope and an audiometer. For screening purposes, it performs a pure tone sweep across frequencies of 500, 1000, 2000, and 4000 Hz and the patient reports whether tones were heard. Background noise in the examination room must be kept to a minimum. There is some debate as to what intensities and frequencies should be used in the screening protocol to maximize the test’s sensitivity and specificity (25). The use of self-administered questionnaires regarding the patient’s hearing handicap in certain situations is a simple and inexpensive approach to screening hearing. The hearing handicap inventory for the elderly-screening version is a 10-item, five-minute questionnaire that measures the degree of social and emotional hearing handicap (26). The American Academy of Otolaryngology devised the five minute hearing test, which is another self-administered questionnaire that can be used for screening purposes (27). These self-reported screening tests assess not only the patient’s self-perceived hearing loss but also their motivation for hearing care. Comprehensive Audiologic Examination The routine audiologic test battery consists of pure tone and speech testing, static immittance, and acoustic reflexes. Word recognition scores, used for diagnostic purposes, also play a role in the aural rehabilitation program. If word recognition ability at audible levels is poor, then counseling must include realistic expectations of the results of amplification. Although such a patient will benefit from amplification, the patient usually must rely more heavily on visual cues and/or assistive listening devices. A common challenge to rehabilitation in the hearing-impaired population is a reduced dynamic range, referring to the range between the thresholds of audibility and loudness discomfort. In age-related hearing loss, the elevated threshold of audibility contributes to the reduction of the dynamic range. The relationship between the change in audibility threshold and loudness discomfort level, however, is not linear due to recruitment. Recruitment is abnormal loudness gain for suprathreshold stimuli. In other words, sounds increase from very soft to uncomfortably loud rapidly with increasing stimuli. Determination of the elderly individual’s loudness

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discomfort level when fitting a hearing aid or any other type of assistive listening device is important to ensure that the maximum output of the hearing aids does not exceed this level. A salient characteristic of the elderly hearing-impaired listener is the decreased understanding of speech in the presence of background noise. In a study by Frisna and Frisna (28), a group of younger listeners and a group of elderly listeners with equivalent audiometric sensitivity completed a variety of speech recognition tasks in quiet and in background noise. It was found that although the elderly group’s hearing sensitivity did not differ from the younger listeners, they performed significantly worse on speech recognition tasks in background noise than their younger counterparts. There are several speech recognition tests that quantify the difficulty the elderly patient is likely to experience with background noise, such as the hearing in noise test (HINT), and a shortened version of the speech in noise test (QUICKSIN). Performance on these tests may help guide the aural rehabilitation program. TREATMENT OPTIONS Testing is completed when the type, degree, and configuration of the hearing loss is defined for each ear separately; the individual’s ability to understand words in quiet and in noise is documented; and the elderly individual’s communication needs, challenges, and expectations are understood in the context of the individual’s communication environment. All patients who exhibit a hearing loss, regardless of the severity, are candidates for an aural rehabilitation program and/or amplification. The aural rehabilitation program can be divided into four sections (Table 1): the selection of hearing aids or other listening devices (this session is called the hearing aid evaluation), the hearing aid fitting, orientation, and counseling. During the hearing aid evaluation, many decisions regarding hearing aid features need to be made. Table 2 contains a list of all of the up front decisions that the elderly individuals, their family, and the audiologist need to consider. The following sections focus on five of the major considerations. These include style of hearing aid, hearing in noise, signal processing, use of the telephone, and safety.

Table 1 Four Components of an Aural Rehabilitation Program Hearing aid selection and evaluation Selection Monaural versus binaural Omnidirectional versus directional microphones Hearing aid style

Hearing aid fit Verification Validation

Telecoil Signal processing Use of other ALDs Abbreviation: ALDs, assistive listening devices.

Hearing aid orientation Insertion and removal Care and maintenance Use of volume control and/or memory push button Use of hearing aid programs/memories

Counseling and training Expectations Communication strategies Understanding the hearing aid features Listening practice

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Table 2 Up Front Considerations That Must be Made Prior to Ordering the Hearing Aid Style of hearing aid Monaural versus binaural amplification Microphone type Earmold type, earmold length, earmold venting Sound channel characteristics Bandwidth of the instrument Receiver type Output limiting Volume control Batteries (battery door locks) Signal processing Number of signal processing channels Multimemory Ability to fine tune (programmable) The decisions are made based on empirical evidence, audiologist experience, and patient and family preference.

Hearing Aid Style The common styles of hearing aids are behind the ear (BTE), in the ear (ITE), in the canal (ITC), and completely in the canal (CIC) (Fig. 2). In selecting the style (or the size) of the hearing aid, the audiologist must consider the individual’s dexterity, ear canal size and texture, history of cerumen accumulation, and degree of hearing loss. The BTE hearing aid has the most flexibility. BTE hearing aids have much more room for circuitry and options. The size and shape of the ear canal does not

Figure 2 Four styles of hearing aids (clockwise from bottom left): behind the ear, in the ear, in the canal, completely in the canal.

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Figure 3 Flowchart of activity for a patient complaining of hearing loss.

interfere with the choices of hearing aid options for the patient using a BTE hearing aid. BTEs can provide the necessary gain to accommodate hearing losses ranging anywhere from mild to profound. BTE hearing aids are coupled to the ear with an earmold. By making specific changes to the earmold (length of tubing, belling of

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sound bore, vent sizes, etc.), a particular frequency/gain enhancement can be created. Patients usually report that these acoustic modifications sound more natural than electronic modifications accomplished through hearing aid circuitry. Other advantages of having a standard hearing aid coupled to a custom earmold include the ability to attach a loaner hearing aid to the user’s earmold should the hearing aid need repair or service. Another advantage of BTE hearing instruments is that care and maintenance is less problematic than with other types of hearing aids. With custom hearing aids that fit completely inside the ear canal and concha, the electronics, particularly the receiver, are vulnerable to cerumen blockage and damage from heat and moisture. Because the electronics of the BTE hearing aid are housed external to the ear canal, it is not vulnerable to these elements found in the ear canal. The earmold can accumulate cerumen blockage, but the patient simply brushes away the visible cerumen or disconnects the earmold from the hearing aid to soak the mold in a cup of warm water. If the elderly patient has a history of cerumen accumulation, a BTE hearing aid should be considered. Insertion, removal, and manipulation (changing the battery) of BTE instruments are relatively easy. Because it is bigger, it may be beneficial to an elderly individual who experiences problems with manual dexterity. Some patients find the bulkiness of this hearing aid a drawback to its use. However, with appropriate hair styling this aid can be very discreet. The earmold is made of a translucent plastic that blends in with the skin tone of the patient and the hearing aid portion is tucked neatly behind the ear. ITE and ITC hearing aids are limited in their flexibility due to space limitations. A patient’s ear canal may be too small to accommodate certain options such as a telecoil or directional microphones. These hearing aids typically fit a hearing loss range of mild to moderate. Due to the close proximity of the microphone and receiver, these hearing aids are more vulnerable to feedback, especially if the hearing loss is more severe. Feedback occurs when amplified sound ‘‘feeds’’ back into the microphone to be reamplified. Proper fit of these custom products is imperative. Manipulation of these custom hearing aids may be more difficult for the elderly patient. The batteries in these hearing aids may be smaller in size and are not appropriate for someone with poor visual acuity and/or manual dexterity. However, several modifications can be made to reduce problems due to dexterity. A removal string can be added to ITE or ITC hearing aids for easier removal of the instrument from the ear. The volume control wheel can be stacked, making it more pronounced for easier manipulation. In some of the more advanced technology, the audiologist is able to enable and disable the volume control. This feature is very useful if you find an aging patient who is confused by the volume control. Rather than send the hearing aid back to have the volume control removed, it simply can be disabled. There are individual differences in manipulation. For some older adults, insertion of one piece (the ITE) is easier than the two-piece BTE, which requires an extra step of looping the hearing aid behind the ear. A CIC hearing aid may be the least visible of all the hearing instruments. A CIC hearing instrument fits deeply inside the patient’s ear canal. The goal of a CIC is to take advantage of the acoustic properties of the external ear. However, it is not the best recommendation for an elderly individual for a number of reasons. It is the most limited in terms of flexibility and only can meet the needs of hearing loss in the mild-to-moderate range. There is no space for any additional options. Because of deep insertion, it is more prone to cerumen blockage, therefore requiring more cleaning and maintenance on the patient’s part. For the elderly patient, extra visits into the clinic for repairs and frequent cerumen removal may be very difficult.

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Table 3 Pros and Cons of Hearing Aid Styles BTE

ITE

ITC

CIC

Flexibility— hearing loss fitting range Flexibility— number of options Manipulation Care and maintenance

Mild to profound

Mild to severe

Mild to severe

Mild to moderate

Very flexible

Dependent on size of ear canal Can be difficult Problems with earwax blockage

Dependent on size of ear canal Can be difficult Problems with earwax blockage

No options

Cosmetic

Depends on hairstyle

Visible

Less visible

Easy Minimal

Difficult A significant problem with earwax blockage Least visible

Abbreviations: BTE, behind the ear; ITE, in the ear; ITC, in the canal; CIC, completely in the canal.

If a CIC is used, a wax guard system that the individual can replace on their own in order to avoid hearing aid repairs should be considered. The CIC is likely to be more difficult to insert for the elderly patient. The battery size is very small, so routine manipulations such as changing the battery are more difficult. There also may be issues of occlusion effect with the CIC instruments. The occlusion effect is said to occur when patients state that their own voice sounds ‘‘hollow’’ or ‘‘in a barrel’’ due to the hearing aid occluding the ear canal. This effect is typically solved by venting the hearing aid or earmold so that the user does not feel so ‘‘plugged up’’ by the hearing aid. Due to the small size of a CIC hearing aid, adequate venting is not possible. Selection of hearing aid style will be influenced by patient preference. Some patients will have a preconceived notion of what hearing aid style they want. It is important that the audiologist and patient compromise a few features and options in order to select an amplification device that the individual will wear. If the patient is forced to purchase an undesirable hearing aid style, then the hearing aid may not be used at all. The best hearing aids are the ones that the patient will wear. Table 3 is a comparison of hearing aid styles highlighting their pros and cons for the aging adult. Hearing in Noise An estimated 25% of hearing aid users are not wearing their hearing aids due to the negative effects of background noise (29). Due to the nature of age-related hearing loss, simple amplification is inadequate to provide maximum intelligibility in noise. Some of the solutions include binaural amplification, directional microphone technology, noise reduction circuitry, and the use of assistive listening devices. Binaural Amplification A significant advantage of binaural hearing is noise suppression since two ears are necessary to efficiently separate the speech signal from surrounding background noise (30). Binaural amplification provides the user with a 2–3 dB signal-to-noise ratio enhancement (31). It is critical to the success of the aural rehabilitation program to

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do everything possible to enhance the signal-to-noise ratio; hence, binaural amplification should be recommended and encouraged for most patients with presbycusis. In 1980, Dirks and Wilson (32) noted that sounds presented binaurally are perceptually 3 dB louder than the actual level due to binaural summation. This is advantageous in that less gain is needed to achieve the same perception of loudness, resulting in less feedback. In addition to noise suppression and binaural summation, binaural hearing is critical to sound localization via comparison of the intensity and temporal cues from each ear individually. Binaural amplification aids the user’s ability to localize sound, which may be important for the safety of the elderly individual in certain situations. Binaural interference is a phenomenon that occurs in some elderly patients who exhibit a large asymmetry in word recognition scores between the right and left ear and is a contraindication for binaural amplification (33). Word recognition scores should be tested individually and binaurally. If the binaural word recognition score is significantly poorer than that of the better ear, then binaural amplification may be inappropriate. Directional Microphones Directional microphones to improve performance in noise recently have been introduced and may benefit some individuals (34–37). All hearing aids dispensed have an omnidirectional microphone, which collects sounds from any directional source and passes them through to the amplifier without relative delay. A hearing aid with a directional microphone involves the use of an additional rear-facing microphone. Input from the additional rear microphone is delayed so that the signal from behind the person is cancelled. Thus, in a directional microphone hearing aid, sounds in front of the wearer are amplified, but the sounds behind them are attenuated. This type of technology provides significant improvement in the presence of background noise, provided that the noise is behind the patient and the signal is in front. The magnitude of the cancellation effect of the directional microphones increases as the listening environment becomes more unfavorable, even with the level of the noise being louder than the level of speech (34). Hearing aids that have directional microphone technology are equipped with a push button or a switch so that the user can turn the directional program on and off. In quiet listening environments, the user should have the hearing aid switched to omnidirectional mode so that all sounds are amplified regardless of location. Once the listener enters a noisy situation, such as a restaurant, the user can either press the push button or a switch to change into the directional microphone program. For elderly patients with manual dexterity handicaps, a remote control can be ordered to change between the programs stored in the hearing aid. Recently, automatic microphone technology has emerged. Automatic microphones switch back and forth between omnidirectional and directional modes automatically based on the sound environment. This technology is advantageous for the elderly population in that it is more hands free. However, the patient can override this automatic directional program by manipulating the push button or remote control. This may be important in situations such as crossing a busy street. The hearing aid will consider this ‘‘noisy’’ and switch into the directional setting, but for safety the individuals may want to hear all around them. A proper and thorough hearing aid orientation is critical to the use and benefit of directional microphones. The elderly patient has to understand the use of the multiple programs stored in the hearing aid and how to manipulate or change them via the

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push-button or remote control. In addition, the aging patient needs to understand that they must orient themselves so that the noise is predominantly behind them and the speaker in front of them in order for the directional microphones to be of benefit. Noise Reduction Circuitry Noise, when analyzed spectrally and temporally, has a broad frequency band and is steady state or constant. In contrast, the acoustic properties of conversational speech are frequency specific and dynamic. Hearing aid engineers have used these contrasting properties to design noise reduction algorithms that will cancel or reduce steadystate sounds while maintaining the amplification of dynamic stimuli. For example, the sound of an air conditioner fan is a steady-state background noise—its intensity level and frequency band remain constant. A noise reduction circuit will cancel this noise so that it is not amplified by the hearing aid. Although noise reduction circuitry has not been shown to improve user understanding in noise, it may decrease the annoyance of the noise, which could lead to less effort expended in communication situations and/or less fatigue. Assistive Listening Devices Assistive listening devices (ALDs) are a family of instruments that can be either coupled to a hearing aid or used alone to assist the user in awareness and discrimination of speech and environmental sounds. Elderly patients with a profound hearing loss or very poor word recognition scores may need to rely on ALDs for not only maximum speech intelligibility in noise, but for safety concerns as well. Some ALDs use a frequency-modulated radio signal (FM signal) that is coupled directly to the hearing aid via a ‘‘boot’’ (for a BTE hearing aid) or through an electromagnetic neckloop (for a BTE or ITE hearing aid). An FM system consists of an external microphone that is worn by the speaker and transmits the signal to the boot or neckloop. The hearing aid must be set to receive only the FM signal from the microphone or other assistive device such as those used in public places (e.g., churches, concert halls, and theaters). FM systems provide the greatest signal-tonoise ratio enhancement in that the hearing aid only receives what is transmitted from the microphone, which is inches away from the speaker’s mouth since the microphone in the hearing aid is disconnected. Other ALDs include amplified telephones, infrared television amplifiers, and various alerting systems. These systems may be vital in ensuring the elderly individual’s safety. Smoke detectors, doorbell ringers, and personal paging systems may alert an individual through a visual (flashing light), auditory (louder signal), or tactile (vibration) signal. ALDs may be somewhat cumbersome, so it is important that the most appropriate device be selected for the individual’s needs and lifestyle.

Signal Processing The basic components of a hearing aid are the microphone, amplifier, and the receiver. The analog electronic signal processing circuit within the amplifier divides the signal into different frequency bands (the number of these bands varies with the complexity of the circuit), which can be manipulated by changing the gain or the output characteristic of each band. These settings are done in the office by coupling the hearing aids to a computer and programming them with the appropriate software.

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Within the past decade, there have been many significant changes in the electronic signal processing of the hearing aid with the advent of digital signal processing (DSP). Analog signals are converted to digital signals, which permits a nearly infinite number of possible signal alterations. While analog signal processing is limited to three bands or less for frequency shaping and compression, DSP technology can divide the signal into as many as 20 channels for processing. Thus, DSP technology has much more flexibility than analog technology (38). As DSP technology continues to improve, more patients will benefit from the newer technology. Digital hearing aid sales comprised only 7% of the total number of hearing aid sales in 1998 whereas in 2002, 42.7% of hearing aid sales were digital technology (39). It is predicted that all hearing aids dispensed soon will be digital. It is important to understand the relationship between DSP technology and hearing aid user performance. The primary predictor of speech recognition performance is audibility (40). Both analog and digital signal processing circuits have the capabilities to make the acoustic signal audible to the hearing aid user. Valente et al. (41) showed that performance on speech recognition tasks in noise were not significantly different when users were wearing either analog or digital hearing aids. Other research studies have supported this finding (42). Kochin (43) surveyed 500 individuals using DSP technology and 418 controls and found that DSP hearing aids receive an overall higher satisfaction rating. DSP technology also facilitates reduced feedback through feedback cancellation systems, expansion or gain reduction to reduce low-level noise, noise reduction algorithms, and automatic microphone technology.

Use of the Telephone The telephone is an integral part of society and in many situations, the telephone may be the only connection between the elderly individual and family members or friends. Hearing aid users can use the telephone by either placing the receiver over the microphone or by activating a telecoil built into the hearing aid. A telecoil is a magnetic coil inside the hearing aid that picks up the electromagnetic field emitted from the receiver of the telephone (and from FM systems with neckloops) and amplifies the signal. Telecoils can be activated by a push button on the hearing aid, which, when pressed will turn off the microphone of the hearing aid and activate the telecoil. For elderly individuals with manual dexterity problems, the telecoil can be activated automatically by placing a telephone receiver directly on top of a hearing aid equipped with an automatic telecoil. The magnet in the telephone automatically switches the internal magnet in the hearing aid that engages the telecoil receiver. Once the phone is removed, the hearing aid will automatically change back to the regular microphone setting. The advantage of the telecoil is that since the microphone of the hearing aid is deactivated, feedback is eliminated when using the telephone. Some elderly individuals are unable to use the telephone with the hearing aid and often benefit from the use of amplified telephones. In order to use this option, however, the hearing aid must be removed and then replaced at the end of the telephone conversation.

Severe Unilateral Hearing Loss An individual with a nonfunctioning ear along with a normal (or near-normal) hearing ear may experience difficulty localizing, hearing in noise, and hearing when

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communication is directed to the nonfunctioning side. Many individuals with this condition use a variety of communication strategies and environmental manipulations to accommodate this situation, but some patients will benefit from specific strategies. There are three potential solutions: (i) contralateral routing of signal (CROS) hearing aid, (ii) a bone-anchored hearing aid (BAHA) implanted on the nonfunctioning side, and (iii) a powerful conventional hearing aid in the nonfunctioning ear (transcranial CROS). Solution one is configured with a microphone pick up on the nonfunctioning side and the signal is transmitted via a cord or FM signal to a hearing aid case anchored on the normal ear. The signal is routed into the normal ear through an open ear canal fitting so the same ear can receive input normally from the ‘‘good’’ side. Solutions two (BAHA) and three (transcranial CROS) operate on the same principle. The signal is picked up on the nonfunctioning side and is delivered to the normal hearing cochlea via bone conduction. Elderly patients with unilateral hearing loss may become aware of communication difficulty sooner than one would expect based on the reduced thresholds in the previously normal hearing ear. These individuals may be fully adapted to being a monaural listener and may only want improved performance in the mildly impaired ear rather than pursuing some type of CROS or transcranial solution. An alternative is a binaural contralateral routing of signal (BICROS) aid. Middle Ear Implantable Hearing Aids Several middle ear implantable hearing aids have been developed in the past several years. Some of these are partially implantable and others are designed to be fully implantable. The manufacturers of these instruments claim reduced feedback (high-pitched sound when amplified sound reaches the microphone and is reamplified) due to implantation, but the actual results have been varied. One would recommend a trial with traditional binaural amplification prior to recommending this type of instrument for the geriatric patient.

Cochlear Implant An individual with severe to profound sensorineural hearing loss is a cochlear implant candidate. Candidacy for implantation is determined through a battery of performance tests and a hearing aid trial. The most common geriatric patient who would be a cochlear implant candidate would be an individual who suffered sudden, severe-to-profound, permanent sensorineural hearing loss as opposed to the individual who has functioned with this type of hearing loss over their lifetime.

Hearing Aid Fitting During the hearing aid fitting, verification and validation of the hearing aid performance must be measured. This can be accomplished through a Real-Ear1 measurement, which is a probe microphone system that measures the sound pressure level at the eardrum. Real-Ear verification is used to fine-tune the hearing aid until the hearing aid’s output falls within this range so that soft sounds are audible and loud sounds are not uncomfortably loud. Validation ensures that hearing aid performance meets the needs of the patient. This is obtained through the use of subjective questionnaires that look at handicap,

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disability, and satisfaction with hearing aids (44). The score tells the audiologist how much benefit the patient perceives from the hearing aids.

Hearing Aid Orientation The elderly patients and their family members must understand how to use and take care of the hearing devices properly. The orientation session consists of instructions of insertion and removal, how and when to change the battery, how and when to change the hearing aid program designed for listening in background noise, a demonstration of telephone use, etc. (45). Documentation is given to the patient as well as the family or the caretaker. Table 4 provides a checklist of items that should be included in the orientation. There are a number of helpful accessories for the aging patient such as ‘‘Fun Tak1’’ to hold the hearing aid and an extending magnet to capture batteries that may have fallen or to pick up the whole hearing aid if it has fallen and still has the battery in it. Moisture removal with a dehumidifier kit can add life to the hearing aid and greatly reduce repairs. Repairs may be a larger problem for the older patient who may find it difficult to travel to the clinic repeatedly. Other devices include a line to clip the aid to the shirt collar to reduce the possibility of loss and a battery tab to assist in battery insertion.

Counseling and Training The new hearing aid wearer is adjusting to both how the hearing aids feel and how the world sounds and may not like the sound quality until there has been a period of adaptation. Often patients are aware of all sounds such as the sound of shoes on the floor, the refrigerator hum, etc., which creates an uncomfortable sensation. Several weeks are required to accommodate to this new sensation. Kochkin (46) reports that patients received greater satisfaction with their new hearing aids if they were provided with one to two hours of counseling regarding realistic expectations, and the use and care of the hearing aids. Abrahms et al. (47) reported that in a group of aging adults, hearing aid fittings plus aural rehabilitation (counseling) was more cost effective than hearing aid fittings alone.

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REFERENCES 1. Pastor PN, Makuc DM, Reuben C, Xia H. Chartbook on Trends in the Health of Americans. Health, United States. Hyattsville, Maryland: National Center for Health Statistics, 2002. 2. National Center for Health Statistics. (www.cdc.gov/nchs/) 3. National Institute on Deafness and Other Communication Disorders. (www.nicdcd. nih.gov.) 4. Schow R, Nerbonne M. Hearing levels among elderly nursing home residence. J Speech Hear Res 1980; 45:124–132. 5. American Speech-Language-Hearing Association. (www.asha.org.) 6. Desai M, Pratt LA, Lentzner H, Robinson KN. Trends in vision and hearing among older Americans. Aging trends No. 2. Hyattsville, Md.: National Center for Health Statistics, 2001. 7. Keller BK, Morton JL, Thomas VS, Potter JF. The effect of visual and hearing impairments on functional status. J Am Geriatr Soc 1999; 47:1319–1325. 8. Jamieson JR. The impact of hearing impairment. In: Katz J, ed. Handbook of clinical audiology. 4th ed. Baltimore: Williams & Wilkins, 1994:596–615. 9. Carabellese C, Appollonio I, Rozzini R, et al. Sensory impairment and quality of life in a community elderly population. J Am Geriatr Soc 1993; 41:401–407. 10. Palmer CV. Improvement of hearing function. In: Huntley RA, Helfer KS, eds. Communication in later life. Boston: Butterworth-Heinemann, 1995:181–223. 11. Bess FH, Lichtenstein MJ, Logan SA, Burger MC, Nelson E. Hearing impairment as a determinant of function in the elderly. J Am Geriatr Soc 1989; 37:123–128. 12. Mulrow C, Aguilar C, Endicott J, et al. Association between hearing impairment and the quality of life of elderly individuals. J Am Geriatr Soc 1990; 38:45–50. 13. Weinstein B, Ventry I. Hearing impairment and social isolation in the elderly. J Speech Hear Res 1982; 32:593–599. 14. Chmiel R, Jerger J. Some factors affecting the assessment of hearing handicap in the elderly. J Am Acad Audiol 1993; 4:249–257. 15. Gates GA, Karzon RK, Garcia P, et al. Auditory dysfunction in aging and senile dementia of the Alzheimer’s type. Arch Neurol 1995; 52:626–634. 16. Uhlmann R, Larson E, Rees T, Koepsell T, Duckert L. Relationship of hearing impairment to dementia and cognitive dysfunction in older adults. JAMA 1989; 261:1916–1919. 17. Peters CA, Potter JF, Scholer SG. Hearing impairment as a predictor of cognitive decline in dementia. J Am Geriatr Soc 1988; 36:981–986. 18. Durrant J, Gilmartin K, Holland A, Kamerer D, Newall P. Hearing disorders management in Alzheimer’s disease patients. Hear Instrum 1991; 42:32–35. 19. Palmer CV, Adams SW, Bourgeois M, Durrant, JD, Rossi M. Reduction in caregiveridentified problem behaviors in patients with Alzheimer disease post-hearing-aid fitting. J Speech Lang Hear Disord 1999; 42:312–328. 20. Willott JF. Anatomic and physiologic aging: a behavioral neuroscience perspective. J Am Acad Audiol 1998; 7:141–151. 21. Stach B, Spretnjak M, Jerger J. The prevalence of central presbycusis in a clinical population. J Am Acad Audiol 1990; 1:109–115. 22. Jerger J, Chmial R, Wilson N, Luchi R. Hearing impairment in older adults: new concepts. J Am Geriatr Soc 1995; 43:928–935. 23. Herbst K, Humphrey C. Hearing impairment and mental state in the elderly living at home. Br Med J Clin Res 1980; 281:903–905. 24. Canestrari R. Paced and self-paced learning in young and elderly adults. J Gerontol 1963; 18:165–168. 25. Schow RL. Considerations in selecting and validating an adult/elderly hearing screening protocol. Ear Hear 1991; 12:337–348.

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26. Ventry IM, Weinstein BE. Identification of elderly people with hearing problems. ASHA 1983; 7:37–42. 27. American Academy of Otolaryngology—Head & Neck Surgery. The five minute hearing test. AAO-HNS Bull 1990; 9:43. 28. Frisna DR, Frisna RD. Speech recognition in noise and presbycusis: relations to possible neural mechanisms. Hear Res 1997; 106:95–104. 29. Kochin S. MarkeTrak V. ‘‘Why my hearing aids end up in the drawer’’: the consumer’s perspective. Hear J 2000; 53(2):34,36,39–42. 30. Valente M. Binaural amplification: part II. Audecibel 1984; 12:10–14. 31. Hawkins D, Yacullo W. Signal to noise ratio advantange of binaural hearing aids and directional microphones under different levels of reverberation. Speech Hear Disord 1984; 49:278–286. 32. Dirks D, Wilson R. Binaural hearing in sound field. In: Libby ER, ed. Binaural Hearing and Amplification, Vol. 1. Chicago: Zeneron, 1980:105–122. 33. Jerger J, Silman S, Lew H, Chimel R. Case studies in binaural interference: converging evidence from behavioral and electrophysiologic measures. J Am Acad Audiol 1993; 4:122–131. 34. Valente M, Sweetow R, Potts LG, Bingea B. Digital versus analog signal processing: effect of directional microphone. J Am Acad Audiol 1999; 10:133–150. 35. Agnew J, Block M. HINT thresholds for a dual-microphone BTE. Hear Rev 1997; 4(9):26, 29–30. 36. Ricketts T. Impact of noise source configuration on directional hearing aid benefit and performance. Ear Hear 2000; 21:194–205. 37. Ricketts T, Dhar S. Comparison of performance across three directional hearing aids. J Am Acad Audiol 1999; 10:180–189. 38. Agnew J. Amplifiers and circuit algorithms for contemporary hearing aids. In: Valente M, ed. Hearing Aids: Standards, Options, and Limitations. 2nd ed. New York: Thieme, 2002:101–142. 39. Kochkin S. MarkeTrak VI. Ten year customer satisfaction trends in the US hearing instrument market. Hear Rev 2002; 9(10):14–25,46. 40. Humes L, Roberts L. Speech-recognition difficulties of the hearing-impaired elderly: the contributions of audibility. J Speech Hear Res 1990; 33:726–735. 41. Valente M, Fabry DA, Potts LG, Sandlin RE. Comparing the performance of the widex senso digital hearing aid with analog hearing aids. J Am Acad Audiol 1998; 9:342–360. 42. Newman CW, Sandridge SA. Review of research on digital signal processing. In: Valente M, ed. Hearing Aids: Standards, Options, and Limitations. 2nd ed. New York: Thieme, 2002:347–381. 43. Kochin S. Customer satisfaction with single and multiple microphone digital hearing aids. Hear Rev 2000 November:24–34. 44. Cox RM, Alexander GC. The abbreviated profile of hearing aid benefit. Ear Hear 1995; 16:176–186. 45. Mormer E, Palmer C. A systematic program for hearing aid orientation and adjustment. In: Sweetow R, ed. Counseling for Hearing Aid Fittings. San Diego, CA: Singular Publishing, 1999. 46. Kochkin S. Factors impacting consumer choice of dispenser and hearing aid brand; use of ALDs and Computers. Hear Rev 2002; 9(12):12–23. 47. Abrahms H, Chisolm T, McArdle R. J Rehabil Res Dev 2002; 39(5):549–558.

ANNOTATED BIBLIOGRAPHY Bogardus ST, Yeuh B, Shekelle PG. Screening and management of adult hearing loss in primary care: clinical applications. JAMA 2003; 289(15):1986–1990.

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This is an excellent, comprehensive, critical review of the subject of adult hearing screening. Campbell K. Essential audiology for physicians. San Diego: Singular Publishing Group, 1997 This text is aimed at the practicing physician and provides good detail related to audiology while being easily read and well organized. Palmer CV. Improvement of hearing function. In: Huntley RA, Helfer KS, eds. Communication in later life. Boston: Butterworth-Heinemann, 1995:181–223. This chapter provides a great deal of detail regarding modifications to procedures in audiology appropriate for the older adult. Yueh B, Shapiro N, MacLean CH, Shekelle PG. Screening and management of adult hearing loss in primary care: scientific review. JAMA 2003; 289(15):1976–1985. This complements the Bogardus et al. (2003) article with scientific underpinnings.

6 Presbycusis Sidney N. Busis Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

Aging is inevitable. In his classic text, ‘‘Pathology of the Ear,’’ Harold Schuknecht (1) writes, ‘‘Literally, we start aging at the moment of conception and never stop until we die; in the more usual sense, aging starts when growth ceases.’’ Presbycusis, derived from two Greek words—presbus, an old man, and acusis, hearing—is the natural hearing loss that occurs as the auditory system ages. It is the most common type of hearing impairment in humans and is the most common cause of sensorineural hearing loss in the United States (2,3). Presbycusis is the third most prominent chronic medical condition in older Americans after hypertension and arthritis (3,4). It is estimated that 25–40% of the population aged 65 years and older, 40–66% of patients older than 75 years and more than 80–90% of patients older than 85 years are hearing impaired (3–5). The hearing loss that accompanies aging might be called age-related hearing loss because it can be a combination of presbycusis, genetic predisposition to hearing impairment, and noise-induced hearing loss due to occupational or recreational noise exposure or to living for years in our noisy society (called sociocusis). Also age-related changes in other systems may adversely affect auditory function (6). Among others, these changes may include diminished efficiency of the immune system, a tendency to produce antibodies to the body’s own proteins, and cardiovascular changes that can affect the ear and the brain (6,7).

INCIDENCE Understanding and managing presbycusis is important because in the United States there is a large, growing older population. According to the Administration on Aging of the U.S. Department of Health and Human Services, the older population (age 65 or older) numbered 35.6 million in 2002 and is projected to reach 40.2 million in 2010 and 54.6 million in 2020 (8). These older adults have a high risk of developing hearing loss to a degree that can greatly affect their quality of life, and most older people experience further declines in hearing sensitivity over time (5). Loss of hearing is frequently compared to loss of vision. Helen Keller, the famous deaf–blind author and lecturer, when asked to compare the loss of hearing to the loss of vision, reflected that visual loss separates people from ‘‘things’’ and hearing loss separates 77

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people from ‘‘people.’’ This separation from people explains the feeling of isolation that leads to withdrawal from family and friends and can cause serious depression in some people with presbycusis (9). Tinnitus that frequently accompanies presbycusis can also be a cause of major depression (10). Depression associated with hearing loss has been found to be independent of age and socioeconomic status (3). In the elderly, depression is more likely to occur when the individual has distressing medical conditions, as frequently occurs in older people, or when the individual is grieving because of the loss of a loved one or is sad because of other personal or family problems. From the medical perspective, the treatment of hearing loss by the use of hearing aids and assistive listening devices can decrease the morbidity of late-life depression regardless of causation (10).

RECOGNITION Despite the recognized deleterious effects of hearing loss on the quality of life, hearing loss is underdiagnosed; for example, only 9% of internists offer hearing tests to patients over 65 years of age (3). Hearing loss is also undertreated; only 10–25% of patients with aidable hearing loss receive hearing aids (3,6,11). Although the definitive measure of hearing is a complete audiometric examination, screening tests, which can be performed in the primary care physician’s office, are useful for identifying patients who may have an unrecognized significant hearing problem that should be addressed. A subjective screening method for older patients relies on the patient’s own perception of a hearing problem. For this evaluation, a Hearing Handicap Inventory for the Elderly (HHIE) has been developed and has been found to be useful for quantifying hearing handicap in elderly persons (12). A screening version of the Hearing Handicap Inventory for the Elderly, known as the Hearing Handicap Inventory for Elderly-Screening (HHIE-S), a self-administered, 10-item, five-minute questionnaire, has been shown to identify patients with handicapping hearing impairments (Table 1) (3). Scores of 10 and above provide reasonable sensitivity and specificity for significant hearing loss (3). However, Gates et al. (13) in a study of 546 older individuals who had audiometric testing reported that a single global Table 1 Questions from HHIE-S Does a hearing problem cause you to feel embarrassed when meeting new people? Does a hearing problem cause you to feel frustrated when talking to members of your family? Do you have difficulty hearing when someone speaks in a whisper? Do you feel handicapped by a hearing problem? Does a hearing problem cause you difficulty when visiting friends, relatives or neighbors? Does a hearing problem cause you to attend religious services less often than you would like? Does a hearing problem cause you to have arguments with family members? Does a hearing problem cause you difficulty when listening to TV or radio? Do you feel that any difficulty with your hearing limits or hampers your personal or social life? Does a hearing problem cause you difficulty when in a restaurant with relatives or friends? The HHIE-S scores are yes, 4 points; sometimes, 2 points; or no, 0 points, to each. Scores range from 0 (no handicap) to 40 (maximum handicap). Scores of 10 and above provide reasonable sensitivity and specificity for significant hearing loss. Abbreviation: HHIE-S, hearing handicap inventory for the elderly-screening version. Source: From Ref. 3.

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question, ‘‘Do you have a hearing problem now?’’ was more effective than the HHIES in identifying older individuals with unrecognized handicapping hearing loss. Comparing these two screening methods to audiometric examinations in the same patients, Gates found that the HHIE-S had a sensitivity of 35% and a specificity of 94%, and the global question had greater sensitivity, 71%, but lower specificity, 71%. For office screening in the primary care physician’s office, a relatively simple test of hearing is the use of the AudioScope (Welch Allyn) that offers a choice of three screening loudness levels of sound at four frequencies (3). Patients who are found, by any screening method, to have a hearing problem should be referred for an otologic examination and audiometry. Presbycusis usually becomes apparent in the later years of life; however, it may begin to be manifested as early as the third or fourth decade of life. The American National Standards Institute Standard ANSI S3.44-1996 includes tables listing decibels of hearing loss due to age starting in the third decade (14). The International Organization for Standardization Standard ISO 7029 (15) notes that it is well known that sensitivity of the human ear usually falls progressively with age and that the impairment of hearing develops more rapidly for sounds at the high frequencies than at the low frequencies. The Standard notes that the magnitude of this effect varies considerably among individuals. It is further noted that when testing the hearing of persons over 18 years of age, part of any observed hearing loss will probably be associated with age. It cautions that the decrease in hearing may not necessarily be caused by aging alone but by other injurious influences. Hearing losses in decibels at frequencies from 125 to 8000 Hz for different ages, beginning at age 20 through age 70, are listed in the Standard in Annex C. In the table, at the higher frequencies males have greater hearing impairments than females. Schuknecht (1) noted that women have significantly better hearing than men at 2000 Hz and above and surveys have indicated that presbycusis is greater in men than it is in women. This may be attributed to the likelihood that men are generally exposed to more noise in their lifetime, but the reason for the difference is probably more complex. Pathologically, presbycusis may involve three fundamental neural systems: the peripheral auditory system, the central auditory pathways, and the cortical cognitive level (16,17). There is also experimental evidence that the aging middle ear may contribute to age-related hearing loss (2).

PERIPHERAL PATHOLOGY Schuknecht (1) described four types of peripheral auditory pathology that can account for presbycusis: sensory, neural, strial, and cochlear conductive presbycusis. Sensory presbycusis is characterized by a loss of hair cells beginning in the basal turn of the cochlea and extending into the speech frequency area. The criterion for neural presbycusis is loss of 50% or more of the cochlear neurons compared to neonatal normal (1). Strial presbycusis, also called metabolic presbycusis, is associated with loss of 30% or more of the tissue in the stria vascularis (2). Schuknecht postulated that loss of strial tissue affects the quality of endolymph, a change in endolymph that has a detrimental effect on the physical and chemical processes by which energy is made available to the hair cells. There is recent evidence that strial atrophy is the primary cause of cochlear aging (18). The pathology in cochlear conductive presbycusis is thought to be stiffness of the basilar membrane.

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AUDIOMETRIC PATTERNS Audiometrically, sensory presbycusis is characterized by normal hearing or a mild hearing loss in the lower frequencies with a sharp drop beginning at 1000 Hz to a severe loss in the higher frequencies. Clinically, neural presbycusis is characterized by progressive loss of word recognition ability in the presence of stable pure tone thresholds. Audiometrically, neural presbycusis is a high-frequency loss with a more gradual curve from low frequency to high frequency. Audiometrically, strial presbycusis is characterized by a flatter curve with somewhat poorer hearing in the high frequencies and with excellent word recognition scores. It has been reported that poor low-frequency hearing, typical of strial or metabolic presbycusis, is associated with cardiovascular diseases (heart attacks, stroke, intermittent claudication) and that this effect is present in both men and women but is greater in women (7,19). The audiogram of cochlear conductive presbycusis shows a gradually descending pure tone threshold over a range of at least five octaves with a difference of at least 50 dB between the best and worst thresholds and not more than 25 dB difference between any two adjacent frequencies. Schuknecht (1) notes that the diagnosis of a cochlear conductive presbycusis is ‘‘derived by histologic exclusion of any reasonable light microscopic explanation for the gradual descending pure tone threshold type of hearing loss’’ (Fig. 1). Schuknecht and Gacek (20) reported a fifth and a sixth type of peripheral presbycusis, mixed, and indeterminate presbycusis. Based on a survey of the entire collection of 1500 serially sectioned temporal bones at the Massachusetts Eye and Ear Infirmary, they confirmed that the concept of four basic pathologic types of peripheral presbycusis was valid. However, they concluded that (i) sensory cell losses were the least important cause of hearing loss in the aged; these lesions in the basal end of the cochlea caused an abrupt high-frequency loss and often merged with acoustic trauma lesions and could not be differentiated from them; (ii) neuronal lesions are constant and predictable expressions of aging and degrade the capability of word recognition; (iii) atrophy of the stria vascularis occurs as a prominent lesion of aging and principally involves the apical and middle turns of the cochlea and characteristically shows a flat audiometric pattern; (iv) light microscopic studies failed to reveal a pathologic correlate to explain a gradual descending pure tone curve and a cochlear conductive disorder seemed to be the most logical explanation; (v) many aging ears showed a significant involvement of two or more of the four basic types of pathology, resulting in a wide spectrum of alterations in pure tone thresholds and word recognition scores; this was called mixed presbycusis; and (vi) there was an indeterminate group consisting of 25% of cases that did not meet the criteria for any of the four types of presbycusis. It was assumed that the cause was morphological and/or chemical changes that could not be identified on light microscopy study.

CENTRAL AUDITORY PATHWAYS Processing abnormalities occur in the central pathways as the auditory system ages. Research data suggest that central presbycusis is associated with deficits in one or more inhibitory neurotransmitter circuits (6). Normal neural processing involves an interplay and balance between excitatory and inhibitory synapses. Inhibitory

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Figure 1 Examples of audiograms of the four basic types of peripheral pathology that account for presbycusis: (A) sensory, (B) neural, (C) strial, (D) cochlear conductive. Abbreviation: WRS, word recognition score. Source: From Ref. 1.

processes have been shown to play roles in auditory functions such as localization of sound and discrimination in noise (6). A study of central auditory aging supported the hypothesis that age-related changes in GABA (g-aminobutyric acid) transmission in the inferior colliculus in the midbrain alter the balance between excitation and inhibition in the inferior colliculus (21). It was suggested that loss of the inhibitory function of GABA could affect hearing in the elderly (21). Glycine, another important inhibitory neurotransmitter, was found to be significantly decreased in the cochlear nucleus of old mice (6). Identification of specific neurotransmitter changes in parts of the nervous system important for speech processing could possibly lead to the development of pharmacotherapy for some types of age-related hearing loss (21). Age-related changes in the central auditory pathways can result both from the effects of biological aging or from the loss or attenuation of neural input from ears that have peripheral pathology (22). From the behavioral neuroscience perspective, the central effects of biological aging are accompanied by the central effects of the cochlear pathology of aging. Age-related cochlear pathology causes changes in how frequency is ‘‘mapped’’ in the central auditory system and may impair neural inhibition (23). These effects of cochlear pathology on the central auditory system can impair binaural hearing and cause exaggerated ‘‘masking’’ of neural responses

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by noise. These changes in the central auditory system accelerate in the later years of life (23). Psychoacoustic research, on the other hand, has shown that high-level auditory skills such as temporal and binaural processing deteriorate with age irrespective of peripheral hearing loss and that the deterioration becomes more apparent as the complexity of the stimulus increases (6). This deterioration may lead to impaired ability to clearly understand speech, decreased ability to localize sound, and difficulty hearing in the presence of background noise. Auditory brainstem and auditory cortex temporal-resolution dysfunction have been implicated in age-related difficulty in speech processing and in speech recognition in noise (17,24). It has been postulated experimentally that the auditory efferent system plays a role in enhancing signals in noise and, in particular, speech perception in noise (25). It has been shown in the mouse model that distortion product otoacoustic emissions (DPOAE) decrease with age (26). And, it was found that DPOAE were reduced in magnitude when white noise was presented to the opposite ear (25,26). This contralateral suppression of the DPOAE was attributed to activation of the medial olivocochlear system, which has an inhibitory effect on the outer hair cells. Contralateral suppression, measured by reduced DPOAE in the opposite ear, was less in older mice than in younger mice indicating a decline in the efferent system in older animals. This has also been shown in human subjects. Distortion product otoacoustic emissions were smaller in older individuals compared to younger individuals with the same normal hearing and normal middle ear function (25). The decline in the efferent system occurs before the decrease of the DPOAE due to age, and a functional decline in the olivocochlear system with age may explain one reason why the elderly have more difficulty hearing in background noise.

MOLECULAR MECHANISMS The molecular mechanisms underlying aging and their relationship to presbycusis are being studied and an understanding of these mechanisms is emerging. Mitochondria, organelles in the cytoplasm of cells, convert nutrients into energy. Mitochondria have been called the ‘‘power plants’’ in our cells that provide the energy needed to maintain body function and metabolism (27). They have a special type of DNA [mitochondrial DNA (mtDNA)] that may be more vulnerable to damage than the DNA in the chromosomes in the cell nucleus (6). In the respiratory mitochondrial chain, oxygen is reduced to water at the end of the chain. In this enzymatic, oxidative process, oxygen may be partially reduced. There is leakage of single electrons causing partial reduction of oxygen leading to the formation of superoxide. Superoxide is a chemical that is highly toxic to cells; it is one of a class of toxic chemicals called free radicals. Free radicals are chemical species with unpaired electrons; they are reactive because electrons like to pair up to form stable two-electron bonds and so are called ‘‘reactive oxygen species (ROS)’’ (27). Free radicals can also be produced elsewhere, e.g., endogenously by leukocytes to combat pathogens and exogenously by environmental pollutants such as cigarette smoke. Oxidative damage by ROS and other free radicals is neutralized by the human body’s antioxidant defense systems; one important component of these systems is comprised of antioxidant enzymes including, among others, the antioxidant enzyme superoxide dismutase (27). In the elderly, there may be a significant increase in the production of ROS and/or a weakening of the antioxidant defenses; these are changes that can lead to presbycusis (6,28). This process may explain Schuknecht’s postulate about strial presbycusis.

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Among the several theories regarding the aging process, the ‘‘mitochondrial clock theory’’ has been considered the most engaging explanation for age-related hearing loss (28,29). According to this theory hypoperfusion of the cochlear tissue leads to ischemia and the formation of ROS (6) that are highly toxic to the auditory neuroepithelia. The ROS damage mtDNA, which, as noted, may be more vulnerable to damage than the DNA in chromosomes, resulting in specific mtDNA deletions and reduced mitochondrial membrane potential, eventually making the mitochondria bioenergetically inefficient (6,28). These specific mtDNA deletions are also known as the common aging deletion (28,29). Continued research on the molecular mechanisms involved in aging is important because it may lead to effective treatment for presbycusis with antioxidants that neutralize free radicals, dietary supplements, and special diets (6,29). There has been evidence that some compounds slow down the ‘‘mitochondrial clock,’’ and it has been shown that experimental animals fed caloric-restricted diets and antioxidanttreated animals had more acute hearing than controls (23,28,29).

GENETIC FACTORS Although familial hearing loss in humans may be difficult to document in the laboratory because causes other than heredity may contribute to the hearing loss of an older person, epidemiologic study has shown a relatively strong heritability for presbycusis, especially for the strial type with sensitivity loss for low frequencies (6,30). Numerous genes are known to be expressed in the auditory system and some of these may contribute to presbycusis and may determine the severity and the time of onset of age-related hearing loss. But none have been proven to account for presbycusis (6,31). In a mouse model, a non-syndromic age-related hearing loss gene (Ahl) has been identified (6). There is no evidence that this gene or a similar gene is responsible for presbycusis in humans (E. W. Rubel, personal communication, 2004). Gene mutations may account for non-syndromic hearing loss. In the mouse, the gene Cdh23 may predispose to early onset age-related hearing loss dependent on genetic factors including a mitochondrial mutation (32). Mutation in the homologous human gene, CDH23, may contribute to susceptibility to presbycusis (33).

EVALUATION Clinically, age-related hearing loss is the foremost communication problem of the elderly. Presbycusis is characterized by the gradual, insidious onset of diminished hearing, of which the individual is frequently unaware. The hearing loss is often first apparent to family members and friends who urge the patient to seek medical attention. Presbycusis is bilaterally symmetrical and usually first affects the high frequencies, the sounds of consonants such as ‘‘s’’ and ‘‘t,’’ sounds that enhance the intelligibility of speech (9). Many children have high-pitched voices and speak rapidly; although their speech may be easily heard, it is often difficult to understand. This explains the common cry of grandparents, ‘‘I can’t understand my grandchildren!’’ Because of recruitment, people with cochlear hearing losses may have a narrow ‘‘dynamic range of hearing’’ in that when speech is soft they cannot hear it, yet when it is somewhat louder it sounds too loud and is unpleasant and annoying. People with presbycusis usually have trouble hearing in noisy places

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and they may also have trouble localizing sound and difficulty perceiving sudden, rapid changes in speech. Presbycusis is frequently accompanied by high-pitched tinnitus, which occasionally becomes a major problem in addition to the hearing impairment. Although in many cases the diagnosis of presbycusis may seem apparent after a cursory history and physical examination and an audiologic test, there are other causes of sensorineural hearing loss simulating presbycusis so that a complete history and physical examination should be performed. The differential diagnosis for presbycusis includes hearing loss due to occupational or recreational noise exposure, acoustic or physical trauma, heredity and gene mutation, ototoxicity, acoustic tumor, and systemic disease. Conductive and mixed hearing losses due to external and middle ear disease also occur in older people. The history encompasses all aspects of an otologic history including questions about: (i) whether the hearing loss is the same in both ears or greater in one ear; (ii) the onset and progression of the hearing loss; (iii) situations in which it is difficult to hear, such as more trouble hearing in quiet places or in noisy places; (iv) hearing the telephone ring and hearing on the telephone; (v) hearing in a one-to-one conversation; (vi) hearing women’s and children’s voices; (vii) fluctuations in hearing; (viii) the presence and the description of tinnitus, dizziness, balance disturbance, ear fullness, pain, or aural discharge; (ix) prior ear disease or ear operations and ear or head physical trauma; (x) occupational, recreational, or military noise exposure, with or without wearing hearing protection devices; (xi) immediate and extended family history of hearing loss; and (xii) the use of hearing aids. Responses to these questions will help quantify the hearing difficulty the patient is experiencing and may suggest etiologies other than, or in addition to, the aging process. Review of the patient’s social and general medical history is also important, since it may reveal activities, systemic diseases, injuries or medications that can contribute to hearing impairment. The examination consists of examining the head and neck including: (i) the nose, nasopharynx, and pharynx; (ii) the areas about the external ear; (iii) the ear canal and tympanic membrane preferably using a microscope; (iv) the tympanic membrane for mobility with the pneumatic otoscope; (v) the tympanic membrane microscopically while the patient breathes through the mouth and nose to see if there is accompanying movement of the tympanic membrane indicating a patulous eustachian tube; (vi) evaluation of seventh nerve function; (vii) testing the corneal reflex for fifth nerve function; (viii) examining for nystagmus in all positions of the eyes; (ix) auscultation of the head and neck if the patient has objective or pulsatile tinnitus; and (x) office vestibular tests if there is a history of vertigo and a fistula test if there is anything to suggest a perilymph fistula. As with the history, abnormalities on physical examination may suggest etiologies other than, or in addition to, the aging process. For example, there are abnormalities of the ear canals and middle ears that can impair hearing. Dry, hard cerumen is frequently found impacted in the ear canals of older people and should be removed (34). Impacted cerumen can cause a conductive hearing loss and it often impairs the function of hearing aids. Middle ear effusion is not uncommon in the elderly. Audiologic evaluation includes air and bone conduction pure tone threshold tests, speech reception thresholds, and word recognition scores (speech discrimination). Hearing is also tested in a sound field in quiet and in noise and, if the patient has hearing aids, with and without the aids in place. If on audiometric testing there is a question about the relationship between air and bone conduction thresholds, tuning fork tests (Rinne´ and Weber tests) can be useful in interpretation. Audiometry

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is an essential element for diagnosis, for measuring the extent of the patient’s hearing loss, and for evaluating the effectiveness of hearing aids. Tympanometry, as another diagnostic tool, is performed to measure middle ear pressure and compliance and for testing acoustic reflexes. Although special tests of auditory function, distortion product otoacoustic emissions, auditory brain stem responses, and electrocochleography have been shown experimentally and clinically to be abnormal in patients with presbycusis, these tests are not specific enough for the clinical diagnosis of presbycusis to warrant their routine use in the evaluation of patients for presbycusis (35–40). For patients with problematic diagnoses, these tests can be useful. In consideration of the possibility of systemic disease contributing to the sensorineural hearing loss, a battery of screening blood tests is indicated unless the patient’s primary care physician has performed the tests recently, as is frequently the case (Table 2) (41). If there is significant asymmetry in the hearing thresholds of one ear compared to the other or if there has been the recent onset of unilateral tinnitus, the possibility of a acoustic tumor or other central nervous system lesion should be considered and a magnetic resonance image (MRI) of the brain with special attention to the internal auditory canals and cerebellopontine angles should be performed. The vast majority of the elderly who have a hearing loss, have a sensorineural hearing loss. If the patient has a conductive or a mixed hearing loss, the external and middle ears are studied further including consideration of a computed tomography (CT) scan of the temporal bones.

MANAGEMENT The basic principles of the management of presbycusis are education and amplification. An elderly person suspected of having a hearing problem should be encouraged to have a complete otologic examination, including audiometry, and a medical examination to determine if there are any systemic conditions that might affect hearing. Counseling is most important. The persons with the hearing loss must understand what is happening in their auditory systems. They should be shown the Table 2 Screening Blood Tests Complete blood count and differential Platelet count Sedimentation rate Cholesterol (HDL and LDL) Triglycerides TSH Free T-4 HbA1c RPR ANA Rheumatoid factor Otoblot test if the patient has a rapidly progressing sensorineural hearing loss or there is other evidence of autoimmune disease Abbreviations: HDL, high-density lipoprotein; LDL, low-density lipoprotein; TSH, thyroid stimulating hormone; ANA, antinuclear antibody; RPR, rapid plasma reagin.

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Table 3 Guides to Better Hearing Hearing is our most important means of communicating with each other. It is a combination of speaking and listening that can be a relaxed, pleasant exchange, or it can be a frustrating experience. Successful communication is equally dependent on the speaker and the listener. A normal-hearing person may sometimes misunderstand a sentence or a word. This is acceptable to all. However, when a person with a hearing impairment misunderstands a sentence or a word, it is immediately assumed that the listener is to blame for the misunderstanding. It may, in fact, have been due to the manner in which the sentence or words were spoken and so the fault of the speaker. Therefore, these are guides to help both the speaker and the listener. For the speaker When communicating with a person who has a hearing loss, you must be very aware of when and how you talk. If the suggestions listed below are followed carefully, the person to whom you are speaking will understand you better: 1. Always alert the person before you speak. For example, call the person by name first and then when the person looks at you begin to speak. 2. Speak a little more slowly and a little more distinctly, softly accenting important syllables 3. Speak a little louder than usual; however, do not shout. 4. If the person to whom you are speaking does not understand you, do not keep repeating the same words. Rephrase. Say it another way, using different words. 5. Never talk with your back turned to someone with a hearing loss. 6. Never call to or try to talk to a person from a distance that you know or suspect is too far for the person to hear you. 7. Avoid eating, chewing, or covering your mouth with your hands while speaking. 8. Remember that a person who has a hearing loss usually has more difficulty hearing when there is a lot of noise around. Therefore, when talking in a noisy place, try to be even more careful about how you speak. If possible, before speaking, try to reduce the noise level, for example by turning down the radio or television, or moving into a quieter place. 9. Be patient and understanding. For the listener The following suggestions will help you to hear and understand people better: 1. Don’t be embarrassed to tell people that you have hearing problem. Most people will be considerate and be more careful with their speech. 2. Learn to look directly at the speaker’s face. Watch the speaker’s facial expressions and watch the speaker’s lips. Be determined to master ‘‘speech reading,’’ the ability to understand speech better by watching a speaker’s facial expressions and the lip movements. 3. In any situation, especially when in a group, always place yourself so that you can hear best: (a) If you have one ear that is better than the other, always arrange to have your better ear facing the speaker or other source of sound. (b) If there is strong lighting in the room, always place yourself with your back facing the light so that the light falls on the speaker’s face. (c) Try to be about 6 ft from the speaker. This is an ideal distance for watching facial expressions, lip movements, and gestures. 4. Ask a speaker whom you find particularly difficult to hear to speak a little more slowly and distinctly. 5. Make every effort to relax. Strain causes tension and makes it more difficult to hear and to read speech. 6. Maintain good general health and get plenty of rest.

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audiogram and have an explanation of its meaning and what hearing problems might be expected. It is best to have this discussion in the presence of family members. Everyone in the family should be advised as to the best ways to cope with hearing loss and should be given a list of suggestions as to how the patient and the family can use the patient’s hearing most effectively (Table 3). It is just as important

Figure 2 Decision tree for presbycusis.

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for normal-hearing members of the family to know how to speak, as it is for the hearing impaired to know how to listen. Family members can be understanding and tolerant so that there is a harmonious family life or hearing loss can be used as a weapon causing continuous frustration and strife. There is no proven medical or surgical treatment to stop or slow the aging of the auditory system. There are proprietary vitamin, mineral, and antioxidant preparations, and nutritional supplements marketed to help hearing. Although they may be helpful, there is no evidence-based medical treatment. As with many bodily dysfunctions, a healthy lifestyle—proper diet, maintaining a healthy body mass index, nonsmoking, regular exercise, avoidance of loud noise and ototoxic substances and other health risks, and periodic medical checkups—helps the person with presbycusis (6). If the hearing loss is significantly affecting the patient’s quality of life, the patient should be evaluated for a hearing aid and given information about assistive listening devices (41). Although rarely indicated for patients with presbycusis, if a severe-toprofound hearing loss develops, a cochlear implant might be considered (Fig. 2) (41). In summary, age-related hearing loss is a major health problem in the United States, affecting the quality of life of millions of people. For now there is no evidencebased medical treatment; however, there is ongoing active research on the auditory system and in related fields so that hopefully effective medical treatment will be available in the future. In the meantime, early detection and evaluation of hearing loss in the elderly can be beneficial to them and their families leading to counseling and the provision of appropriate amplification by hearing aids and assistive listening devices. REFERENCES 1. Schuknecht HF. Pathology of the Ear. 2nd ed. Philadelphia: Lea & Febiger, 1993. 2. Gratton MA, Vazquez AE. Age-related hearing loss: current research. Curr Opin Otolaryngol Head Neck Surg 2003; 11:367–371. 3. Yueh B, Shapiro N, MacLean CH, Shekelle PG. Screening and management of adult hearing loss in primary care. JAMA 2003; 289:1976–1984. 4. Bogardus ST Jr, Yueh B, Shekelle PG. Screening and management of adult hearing loss in primary care. JAMA 2003; 289:1986–1990. 5. Cruickshanks KJ, Tweed TS, Wiley TL, et al. The 5-year incidence and progression of hearing loss. Arch Otolaryngol Head Neck Surg 2003; 129:1041–1046. 6. Willott JF, Chisolm TH, Lister JJ. Modulation of presbycusis: current status and future directions. Audiol Neurootol 2001; 6:231–249. A comprehensive treatise on presbycusis including discussions of variables related to biologic aging, genetics, noise-induced hearing loss, moderately augmented acoustic environment, neural plasticity and the central auditory system, neural plasticity and hearing aids, socioeconomic and cultural barriers to hearing aid use, lifestyle, medical variables, pharmaceutical interventions for presbycusis, and cognitive variables. 7. Gates GA, Cobb JL, D’Agostino RB, Wolf PA. The relation of hearing in the elderly to the presence of cardiovascular disease and cardiovascular risk factors. Arch Otolaryngol Head Neck Surg 1993; 119:156–161. 8. Administration on Aging. A profile of older Americans. U.S. Department of Health and Human Services, Washington, DC 2003:1–16. 9. McCartney JH, Nadler G. How to help your patient cope with hearing loss. Geriatrics 1979; 34:69–71, 75–76.

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10. Kalayam B, Meyers BS, Kakuma T, et al. Age at onset of geriatric depression and sensorineural hearing deficits. Biol Psychiatry 1995; 38:649–658. 11. Gates GA, Cooper JC Jr, Kannel WB, Miller NJ. Hearing in the elderly: the Framingham cohort, 1983–1985. Part I. Basic audiometric test results. Ear Hear 1990; 11:247–256. 12. Ventry IM, Weinstein BE. The hearing handicap inventory for the elderly: a new tool. Ear Hear 1982; 3:128–134. 13. Gates GA, Murphy M, Rees TS, Fraher A. Screening for handicapping hearing loss in the elderly. J Fam Pract 2003; 52:56–62. 14. The American National Standards Institute. Determination of occupational noise exposure and estimation of noise-induced hearing impairment. American National Standard ANSI S3.44, 1996. 15. International Organization for Standardization. Acoustics—statistical distribution of hearing thresholds as a function of age. 2nd ed. International Standard ISO 7029, 2000–05–01. 16. Cohn ES. Hearing loss with aging. Clin Geriatr Med 1999; 15:14. 17. Frisina DR, Frisina RD. Speech recognition in noise and presbycusis: relations to possible neural mechanisms. Hear Res 1997; 106:95–104. One of the main complaints of people with presbycusis is the inability to understand speech in the presence of background noise. This paper clearly addresses the issue clinically and defines the central auditory dysfunction that may account for this common complaint. 18. Gates GA, Mills D, Nam BH, D’Agostino R, Rubel EW. Effects of age on the distortion product otoacoustic emission growth functions. Hear Res 2002; 163:53–60. 19. Gates GA, Rees TS. Hear ye? Hear ye! Successful auditory aging. West J Med 1997; 167:247–252. 20. Schuknecht HF, Gacek MR. Cochlear pathology in presbycusis. Ann Otol Rhinol Laryngol 1993; 102:1–16. This is a thoughtful and authoritative extension of Schuknecht’s classic work on cochlear pathology in presbycusis. Schuknecht’s chapter on cochlear pathology in Ref. 1 should be reviewed in conjunction with this paper. 21. Caspary DM, Milbrandt JC, Helfert RH. Central auditory aging: GABA changes in the inferior colliculus. Exp Gerontol 1995; 30:349–360. 22. Chisolm TH, Willott JF, Lister JJ. The aging auditory system: anatomic and physiologic changes and implications for rehabilitation. Int J Audiol 2003; 42:2S3–10. 23. Willott JF. Anatomic and physiologic aging: a behavioral neuroscience perspective. J Am Acad Audiol 1996; 7:141–151. 24. Tremblay KL, Piskosz M, Souza P. Effects of age and age-related hearing loss on the neural representation of speech cues. Clin Neurophysiol 2003; 114:1332–1343. 25. Kim S, Frisina DR, Frisina RD. Effects of age on contralateral suppression of distortion product otoacoustic emissions in human listeners with normal hearing. Audiol Neurootol 2002; 7:348–357. 26. Jacobson M, Kim S, Romney J, Zhu X, Frisina RD. Contralateral suppression of distortionproduct otoacoustic emissions declines with age: a comparison of findings in CBA mice with human listeners. Laryngoscope 2003; 113:1707–1713. 27. Frei B. Reactive oxygen species and antioxidant vitamins: mechanisms of action. Am J Med 1994; 97:3A-5S–13S. This is a scholarly overview of the mechanisms of the production of reactive oxygen species in biologic systems and the various antioxidant defense systems that provide protection against oxidative damage to biologic macromolecules.

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28. Seidman MD, Ahmad N, Bai U. Molecular mechanisms of age-related hearing loss. Ageing Res Rev 2002; 1:331–343. An excellent presentation of the various molecular mechanisms underlying age-related hearing loss and a discussion of potential ways to mitigate the effects of aging on hearing. 29. Seidman MD. Effects of dietary restriction and antioxidants on presbyacusis. Laryngoscope 2000; 110:727–738. 30. Gates GA, Couropmitree NN, Myers RH. Genetic associations in age-related hearing thresholds. Arch Otolaryngol Head Neck Surg 1999; 125:654–659. 31. Jennings CR, Jones NS. Review article presbyacusis. J Laryngol Otol 2001; 115:171–178. 32. Noben-Trauth K, Zheng QY, Johnson KR. Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss. Nat Genet 2003; 35:21–23. 33. Siemens J, Lillo C, Dumont RA, et al. Nature Cadherin 23 is a component of the tip link in hair-cell stereocilia. Nature 2004; 428:950–955. 34. Busis SN, Gaca GJ. Removing cerumen: technique, risks, and informed consent. JAMA 1988; 260:99–100. 35. Parham K. Distortion product otoacoustic emissions in the C57BL/6J mouse model of age-related hearing loss. Hear Res 1997; 112:216–234. 36. Lonsbury-Martin BL, Cutler WM, Martin GK. Evidence for the influence of aging on distortion-product otoacoustic emissions in humans. J Acoust Soc Am 1991; 89: 1749–1759. 37. Cilento BW, Norton SJ, Gates GA. The effects of aging and hearing loss on distortion product otoacoustic emissions. Otolaryngol Head Neck Surg 2003; 129:382–389. 38. Rosenhall U, Pedersen K, Dotevall M. Effects of presbycusis and other types of hearing loss on auditory brainstem responses. Scand Audiol 1986; 15:179–185. 39. Boettcher FA. Presbyacusis and the auditory brainstem response. J Speech Lang Hear Res 2002; 45:1249–1261. 40. Oku T, Hasegewa M. The influence of aging on auditory brainstem response and electrocochleography in the elderly. ORL J Otorhinolaryngol Relat Spec 1997; 59:141–146. 41. Marcincuk MC, Roland PS. Geriatric hearing loss. Understanding the causes and providing appropriate treatment. Geriatrics 2002; 57:44–51.

7 Tinnitus in the Elderly Patient Steven M. Parnes Department of Otolaryngology, Albany Medical Center, Albany, New York, U.S.A.

TINNITUS Tinnitus is an otologic symptom experienced by nearly every individual at some time. It is the perception of sound or noise, which originates in the head and not external to the individual. Tinnitus should be characterized not as a disease, but rather, a complex symptom that requires a thorough evaluation with appropriate diagnostic tests for successful management (1). Subjectively, patients may describe tinnitus as either a pure tone or multiple tones that can be high pitched or low pitched. It is often characterized as a ringing, buzzing, roaring, clicking, or hissing sound. Patients may describe tinnitus as pulsatile in nature. While it can be steady or intermittent, patients usually perceive tinnitus more often at night in a quiet environment. Although tinnitus is poorly understood, it is very common. One of the first efforts to determine the prevalence of tinnitus was a national study conducted throughout England in 1978 (2). The study methodology was a questionnaire with 19,000 respondents. Approximately one in six (16–19%) of respondents over the age of 17 had experienced spontaneous tinnitus lasting more than five minutes. While 8% had a type of tinnitus that was bothersome and interfered with sleep or concentration, only 0.5% felt that it severely hampered their ability to have a normal life. In 1996, the National Center for Health Statistics (United States) conducted a survey to study the prevalence of tinnitus (3). With all ages considered, the prevalence was 3%; however, tinnitus was present in 1% of those surveyed under the age of 45, and 9% over the age of 65 (Fig. 1). These results, as well as others, confirm the clinical impression that an increasing incidence of tinnitus correlates with increasing age (1–3). An estimated 40–60 million people in the United States experience tinnitus, the symptom occurring most commonly between the ages of 40 and 70, more in men than in women, and rarely in children (4). In fact, one has stated that nearly the entire population will have this symptom at some time in their lives, with approximately 10 million people finding the symptom to be severe or troubling. Tinnitus is closely correlated with hearing loss. As the population ages, hearing deteriorates and the symptom of tinnitus becomes not only more prevalent but also more bothersome (Fig. 2). A study comparing a tinnitus group to a cohort without tinnitus demonstrated an increasing incidence of hearing loss in the tinnitus group (5). 91

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Figure 1 The prevalence of tinnitus in U.S. the population. Source: From Ref. 4.

The prevalence increases with the severity of hearing loss regardless of the frequency affected by the hearing loss, with tinnitus appearing to stabilize after age 75. The characteristics—and etiologies—of tinnitus can be divided into two major categories, objective or subjective. Objective tinnitus refers to tinnitus that is audible to the physician or another person while subjective tinnitus is apparent only to the sufferer. This definition is rather arbitrary since it is dependent on the fastidiousness of the observer and not on the actual pathophysiology of the patient’s tinnitus. The use of an amplifying stethoscope is more likely to lead to the identification of ‘‘objective’’ tinnitus then routine auscultation in the normal milieu. It is therefore more appropriate to delineate tinnitus into those generated by para-auditory structures versus sensorineural tinnitus, more likely to be encountered (6).

TINNITUS THAT ORIGINATES FROM PARA-AUDITORY STRUCTURES (OBJECTIVE TINNITUS) Tinnitus that can be objectively heard can be further divided into vascular noises or ‘‘clicking.’’ Vascular sounds may be due to venous hum, arterial turbulence, vascular neoplasm, arteriovenous fistulas, intracranial hypertension, or other vascular tumors,

Figure 2 The prevalence of hearing loss in the U.S. population. Source: From Ref. 4.

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and typically are synchronous with the heartbeat. Tinnitus described as clicking in nature is usually secondary to muscular myoclonus, usually from the palate. The astute clinician will differentiate between the patient’s symptoms of random clicking or pulsatile nature of the tinnitus in an effort to elucidate the etiology. Vascular Tinnitus If the patient describes a pulsating type noise, this certainly suggests a vascular etiology even if the examiner is unable to appreciate it (6,7). The tinnitus can be described as a harsh, sharp sound, although if it is of venous origin it would be more like humming machinery with rhythmic accentuations since the sounds are vascular in origin. In addition to doing a careful head and neck exam with particular emphasis on the ear canal and eardrum, it is important to auscultate the patient in various positions. Auscultation should be done with a stethoscope near the eye, the preauricular area, parietal scalp, mastoid, and neck. An instrument likely to be found only in an otolaryngologist’s office is a Toynbee stethoscope, which is a rubber tubing with ear inserts on both ends. This allows the examiner to appreciate the sound more easily. Furthermore, one should note during auscultation whether light neck compression obliterates the sound. Obliteration with light pressure suggests a venous etiology rather than an arterial cause in which greater pressure would be necessary. One should be particularly cautious in the elderly since significant carotid arthrosclerotic disease may be present and compression may trigger an embolic or occlusive event. Otoscopy of the tympanic membrane will rule out middle ear effusion, which can accentuate vascular sounds, and also identify any masses behind the eardrum. If a reddish vascular mass is noted, this could suggest a glomus tympanicum or glomus jugulare. A bluish mass in the hypotympanum suggests a dehiscent jugular bulb, where a reddish one in the anterior middle ear space suggests a dehiscent carotid artery. Employing magnification might be helpful to visualize the possible findings, and if increasing pressure in the ear canal results in blanching of the mass (Brown’s sign), this provides additional confirmation of the presence of a vascular tumor. In addition to an audiogram, tympanometry can be useful in these patients since regular pulsations can be seen on the tracing. Paragangliomas such as glomus jugulare tumors are highly vascular tumors composed of non-chromaffin paraganglioma cells that originate from chemoreceptors or baroreceptors. They are found along the carotid body, jugular bulb, and the middle ear. Patients may have more than one tumor; therefore, further investigation with magnetic resonance imaging as well as arteriography are required if a paraganglioma is suspected. Arteriovenous malformations can also lead to a pulsatile-type tinnitus. In this case, examination of the patient is usually normal. Again, one must be dependent on imaging studies particularly magnetic resonance imaging (MRI), magnetic resonance angiographs (MRAs) and if necessary a four-vessel arteriograph. If this diagnosis is made, then these are usually treated with arterial embolization if possible. Venous hum is another vascular sound that can be differentiated from sounds of arterial origin in that the venous sound is more low pitched and softer and can be obliterated by light pressure on the neck. Treatment is somewhat controversial. Ligation of the internal jugular vein has been performed, but many patients develop recurrent symptoms years later; hence, counseling and reassurance are appropriate for most patients, especially the elderly. In some instances, a magnetic resonance venograph (MRV) may be appropriate to define the abnormality. Pseudotumor cerebri can present in a similar fashion so that a fundoscopic examination to rule out papilledema

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should be performed (7). Pseudotumor cerebri usually occurs in young obese female patients (or in association with anemia or polycythemia) and is unlikely to be encountered in the geriatric population. The exact etiology of pulsatile tinnitus remains unidentified in the majority of elderly patients who present with the complaint. Most will have normal findings on physical examination and unremarkable imaging studies. The internal carotid artery courses within 1–2 mm of the cochlea, so a plausible explanation is that sounds of turbulent blood flow coursing through the neck vessels is transmitted to the inner ear. An ongoing paradigm shift in the management of occlusive vascular disease of the extracranial cerebral circulation may reduce the threshold of intervention for future patients with vascular stenosis. Hence, evaluation of these patients with ultrasonography to rule out significant atherosclerotic disease or stenosis may be warranted. Muscle Contraction Tinnitus Synchronous contraction of the palatal muscles (palatal myoclonus) is a nonvascular cause of objective tinnitus. An involuntary series of rapid, repetitive contractions of the tensor and levator palatini muscles occurring at a frequency of from 60 to 200 times a minute results in an audible click. The click can be heard by both the patient and the examiner and it is synchronous with the movement of the palate. Occasionally, opening the mouth decreases or eliminates the contractions, making diagnosis challenging. Palatal myoclonus is occasionally associated with neurological diseases such as brainstem infarcts and multiple sclerosis, but usually there is no specific etiology identified. Imaging with MRI is necessary and is usually normal in these individuals. Medications that have been used include clonazepam, carbamazepine, and diazepam, all of which have side effects that may be more severe than the primary complaint, particularly in the elderly. Chemodenervation of the involved muscle(s) with botulinum toxin provides some benefit, but care must be taken to avoid palatal paralysis, which can lead to dysphagia. Surgery is rarely indicated and often ineffective (1,6). Patulous Eustachian Tube Patients who suffer sudden weight loss sometimes complain of an ocean roar in the ear that is synchronous with respiration. The sound is worse after the patient wakes up and is up and about during the day, and is not as severe when reclining. Often they will paradoxically complain of a ‘‘plugged’’ feeling, as well as an ‘‘echo’’ when they speak due to transmission of sound through the open eustachian tube. On examination of the ear, one may observe movement of the tympanic membrane during respiration. Tympanometry can also detect an abnormal motion of the eardrum. Fortunately, the symptoms are short lived for most patients, although an occasional patient will have persistent and troublesome symptoms. In these cases, partial or total obliteration of the eustachian tube can be performed; however, this will often lead to middle ear effusion, requiring placement of a patulous eustachian (PE) tube. Effective treatment for this disorder remains elusive (1).

SENSORINEURAL (SUBJECTIVE) TINNITUS This is far and away the most common type of tinnitus encountered. Despite its high prevalence, little is known about the underlying physiologic mechanisms. It is known

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to occur in patients who have suffered insults to the ear such as noise exposure or administration of certain pharmacologic agents. It has also been associated with other otologic diseases such as Meniere’s disease and otosclerosis. Most authorities believe that the illusion of sound is actually generated within the central nervous system (CNS). This is supported by the fact that tinnitus can persist in individuals in whom the auditory nerve has been severed (8). A number of theories exist. One is that deprivation of input to the auditory nervous system releases central neurons from inhibition. Another is that the sensation is due to spontaneous neural activity resulting from ephaptic excitation of one nerve fiber by adjacent fibers or synchronous hair cell discharges (9). Not all attribute the source to the CNS. One theory suggests that outer hair cell damage results in decoupling the stereocilia from the tectorial membrane, leading to an increased discharge rate that evolves from the damaged segment of the outer hair. The lack of a pure animal model has limited the possibility of studying the mechanisms of hearing loss-related tinnitus. None of the theories enumerated above have been substantiated or accepted by all otolaryngologists. Causes and Evaluation The etiology of tinnitus can be divided into the following potential causes: otologic, cardiovascular, metabolic, neurologic, pharmacologic, dental, and psychological factors. The greatest number will be due to otologic factors specifically noise exposure and presbycusis. Other disease processes involving the ear can result in tinnitus, such as Meniere’s disease, otosclerosis, and labyrinthitis. Unilateral tinnitus presents a particular problem since one of the chief complaints of patients with an acoustic neuroma is unilateral tinnitus and hearing loss. An MRI is the diagnostic test of choice. Whether routine testing is warranted in the elderly population is a matter of some debate. All patients should undergo a careful history and physical examination. In particular, the history should focus on the onset and duration of tinnitus and whether there is any history of noise exposure. A tinnitus handicap scale has been developed to document problems associated with tinnitus to determine the impact of the symptom on the patient’s quality of life (QOL). It is apparent that many of these patients suffer from underlying psychiatric disorders and it is important that a clinician is cognizant of this fact (9,13). An audiogram should be obtained in all patients, as well as tympanometry depending on specific circumstances. The audiologist can also determine the loudness or pitch of the tinnitus using tinnitus-matching techniques. This is usually unnecessary unless one is considering a masking device for the treatment (14). Imaging studies may be occasionally warranted in specific cases such as unilateral nature, sudden onset, etc. Diagnosis and treatment of a specific otologic condition may or may not relieve the patient of tinnitus. Cardiovascular problems should be considered, particularly hypertension and anemia, since there seems to be a higher incidence of tinnitus in this patient population. Thyroid dysfunction has been noted as well. Nine percent of patients who have head trauma have tinnitus, so asking about prior head injuries may assist in history taking (1). Postconcussive tinnitus usually occurs in a delayed fashion, beginning one to two weeks after the incident. One must be careful about making a causal association because of risk of secondary gain if litigation is possible. Another common cause of tinnitus is as a side effect of certain drugs and medications. Aspirin and aspirin-like medications are the most common etiologic drugs. Tinnitus caused by these drugs is dose-dependent and is reversible, unlike that caused by the aminoglycosides. Tinnitus may be one of the first symptoms of

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irreversible otologic complications from aminoglycoside use. If unrecognized, patients may proceed to develop permanent hearing loss and loss of vestibular function. This risk has been reduced somewhat with the availability of aminoglycoside blood levels; nevertheless, the symptom of tinnitus mandates careful investigation in these patients. Caffeine may also be a culprit. Some authorities recommend that patients with tinnitus be asked to reduce or eliminate caffeine from their diet. Other drugs that have been implicated are nonsteroid inflammatory drugs and the heterocycline antidepressants (1). A significant number of patients with temporal mandibular joint problems complain of tinnitus. Patients also note a sensation of fullness in the ear. Examination does not reveal any abnormality of the ear canal or eardrum, but palpation of the temporal mandibular joint may elicit pain, tenderness, and crepitus. A history of bruxism as well as joint malalignment are often associated with this complaint. Therapy is directed towards treating the temporomandibular joint (TMJ) abnormalities. Heat, analgesics, and a soft diet are important components of the treatment plan. A bite block may be necessary if significant bruxism is present. It is important to recognize many patients whose QOL is severely affected by tinnitus have underlying psychological disorders. Psychological profiles of these patients demonstrate a pattern of difficulty in coping with the symptom as well as other associated psychological conditions (1,15,16). The question could be asked, ‘‘Does tinnitus cause depression?’’ It is apparent that most of these patients are not severely depressed and can be treated with appropriate counseling, thereby avoiding pharmacologic therapy. The converse question, ‘‘does depression cause tinnitus?’’ is also reasonable. However, there is no evidence to suggest that this is the case. In fact, despite the correlation between the presence of tinnitus and depression, there is no correlation between the intensity of the tinnitus and its effect on QOL (6,13). Management Therapy falls into several categories. First, there is treatment directed at the underlying condition. Specific otologic condition such as labyrinthitis, Meniere’s disease, or otosclerosis can be addressed. Inciting factors such as noise exposure and drugs that can potentially produce tinnitus, such as aspirin and caffeine should be avoided. Other categories include medical therapy, surgical therapy, psychological approaches, and masking. A number of pharmacological approaches have been tried over the years; however, no drug is presently approved by the Food and Drug Administration (FDA) with a specific indication for the treatment of tinnitus. Ginkgo biloba has become popular as a treatment strategy, but when subjected to double blind prospective studies it does not demonstrate effectiveness (6,14). No rigorous study has clearly determined that any medication will benefit patients with tinnitus, in that most studies have been anecdotal and poorly controlled (6,15,17). Patients who suffer from depression or significant anxiety with associated tinnitus may benefit from medical therapy of their depression and/or anxiety. Antidepressants represent the mainstay of this strategy, with specific medication choices, especially in the elderly, being beyond the intent of this chapter (13). No surgical procedure is presently approved for tinnitus. Radical procedures, including trans-section of the cochlear nerve or labyrinthectomy have not been demonstrated to be efficacious (6,15). Surgical therapy directed at specific causes such as Meniere’s disease or hearing loss (cochlear implantation) will occasionally result in

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reduction in tinnitus as an ‘‘extra added benefit’’ of the procedure. A number of studies have demonstrated some degree of tinnitus suppression after cochlear implantation and a modified cochlear implant intended for use in patients with severe tinnitus is under investigation (18,19). There is only a 25% chance of improving tinnitus with cochlear nerve resection, and the severity of tinnitus increased in some patients. Microvascular decompression of the eighth cranial nerve has also been performed by some for severe tinnitus, but this procedure remains controversial (20).

Avoidance of Silence Perhaps the most effective therapy for tinnitus is avoidance of silence. In most instances, patients would have already discovered the effectiveness of background sound in reducing their awareness of tinnitus prior to their evaluation. The exact mechanism of action is unknown, but may be due to providing increased inhibition to central signal generators. Commercial devices are available, although many patients utilize a fan, radio, or some other mechanical or electronic device to assure a constant background sound. Discussion of this option with symptomatic patients is often all the ‘‘treatment’’ that they require.

Tinnitus Masker Tinnitus maskers are devices similar to hearing aids that ‘‘avoid silence’’ by generating and transmitting a constant background sound into the ear(s). Maskers come in three broad categories, ‘‘pure’’ hearing aids, ‘‘pure maskers,’’ and some combination of the two functions in one device. Patients with hearing loss who are fitted with hearing aids will often notice an improvement in their tinnitus. The aids act as ‘‘de facto maskers’’ by amplifying background environmental sounds. Masking devices are also specifically designed for patients with tinnitus. In the past, attempts were made to ‘‘frequency match’’ the masking device with the patient’s tinnitus. However, more recently, ‘‘white noise’’ generators have been used instead, as they can be more easily fitted and have at least equal efficacy. Since most patients with tinnitus also have hearing loss, there is a hearing aid with an integral masking function specifically designed for these individuals (14). When used alone, these masking devices alone may be only transiently effective, with significant noncompliance. Therefore, these devices may be most effective when employed as a part of a ‘‘tinnitus retraining’’ (TRT) program. Tinnitus Retraining Jastreboff and Jastreboff (21) popularized a TRT program in the 1990s that utilizes white noise generators, patient education, and biofeedback. Tinnitus is not eliminated, but patients are taught coping strategies that improve their QOL. Most patients will gain some benefit, but it must be noted that this program is costly, time consuming, and not covered by insurance; hence, only the most highly motivated individuals complete it. In many instances, merely knowing that treatment is available will dramatically reduce the impact of the symptom and improve QOL. TRT (as well as other similar programs that employ patient education and biofeedback) should therefore be considered as part of the armamentarium for patients who are most distressed by the symptom (4,6,13,21).

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CONCLUSIONS Tinnitus is an extremely common symptom, particularly among the elderly. Telling the affected patient to ‘‘just live with it’’ is unhelpful and should be avoided. The elderly symptomatic patient will respond favorably to a concerned and sympathetic approach. A focused history and physical examination, along with audiometric investigation, will identify possible etiologies and facilitate directed therapy for some patients. Education, particularly the necessity to ‘‘avoid silence,’’ and reassurance will benefit all patients to some degree. Fitting with a hearing aid, or masking device, may be beneficial. A few patients will suffer persistent significant reduction in QOL due to tinnitus and depression. Referral for treatment of depression is often necessary. TRT will be effective for most patients with the resources and insight to participate in the program. A considerable amount of information is available online. Excellent patient-centered resources can be found on the websites of the American Academy of Otolaryngology—Head and Neck Surgery Foundation and the American Tinnitus Association (22,23). The most critical point in this chapter is to remember that patients with tinnitus can be treated in a very satisfactory fashion.

REFERENCES 1. Schleuning AJ Jr, Martin WH. Tinnitus. 3rd ed. Head Neck Surg. Chapter 151, 1925–1933. 2. Coles RR. Epidemiology of tinnitus: (1) prevalance. J Laryngol Otol Suppl 1984; 9:7–15. 3. Adams PF, Hendershot GE, Marano MA. Current estimates from the National Health Interview survey, 1996. Hyattsville, Md.: National Center for Health Statistics, 1999. 4. Lockwood AH, Salvi RJ, Burkard RF. Tinnitus. N Engl J Med 2002; 347(9):904–910. 5. Coles RR. Epidemiology of tinnitus: (2) demographic and clinical features. J Laryngol Otol Suppl 1984; 23:441–452. 6. Jastreboff PJ, Gray WC, Mattox DE. Tinnitus and hyperacusis. In: Cummings CW, Fredrickson JM, Harker LA, et al., eds. Otolaryngol HNS, 3rd ed. St. Louis: Mosby, 1998:3198–3222. 7. Sismanis A. Pulsatile tinnitus. Otolaryngol Clin North Am 2003; 36(2):389–402. 8. Moller AR. Pathophysiology of tinnitus. Otolaryngol Clin North Am 2003; 36(2):249–266. 9. Eggermont JJ. Tinnitus: some thought about its origin. J Laryngol Otol Suppl 1984; 9:31. 10. Sweetow RW. Tinnitus reclassified: new oil and old lamp. Otolaryngol Head Neck Surg 1996; 114:582–585. 11. Tyler RS. Does tinnitus originate from hyperactive nerve fibers in the cochlea? J Neurophysiol 1984; 44:76–96. 12. Sewell WF. The relationship between the endocochlear potential and spontaneous activity in auditory nerve fibres of the cat. J Physiol 1984; 347:685–696. 13. Dobie RA. Depression and tinnitus. Otolaryngol Clin North Am 2003; 36(2):383–388. 14. Vernon JA, Meikle MB. Masking devices and alprazolam treatment for tinnitus. Otolaryngol Clin North Am 2003; 36(2):307–320. 15. Parnes SM. Current concepts in the clinical management of patients with tinnitus. Eur Arch Otorhinolaryngol 1997; 254:406–409. 16. Kirsch C, Blanchard E, Parnes S. Psychological characteristics of individuals high and low in their ability to cope with tinnitus. Psychom Med 1989; 51:209–217. 17. Seidman MD, Babu S. Alternative medications and other treatments for tinnitus: facts from fiction. Otolaryngol Clin North Am 2003; 36(2):359–381. 18. Miyamoto RT, Bichey BG. Cochlear implantation for tinnitus suppression. Otolaryngol Clin North Am 2003; 36(2):345–352. 19. House TW, Beackman DE. Tinnitus: surgical treatment. CIBA Fund Symp 1981; d5:xx.

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20. Brookes GB. Vascular-decompression surgery for severe tinnitus. Am J Otol 1996; 17:569–576. 21. Jastreboff PJ, Jastreboff MM. Tinnitus retraining therapy for patients with tinnitus and decreased sound tolerance. Otolaryngol Clin North Am 2003; 36(2):321–336. 22. American Academy of Otolaryngology-HNS Foundation (www.entlink.org) 23. American Tinnitus Association (www.ata.org)

8 Hearing Protection for the Retired Adult Marshall Chasin Musicians’ Clinics of Canada, Toronto, Ontario, Canada

Joanne DeLuzio Graduate Department of Speech-Language Pathology, University of Toronto, Toronto, Ontario, Canada

INTRODUCTION When one typically thinks of hearing protection, images of workers in a factory or on a construction site immediately come to mind. Depending on one’s interest, an image of a rock star may also be conjured up. Research has shown that when it comes to causing hearing loss, music exposure is similar to noise exposure (1). Many issues arise when the topic of noise exposure is raised. Of these, the most common are: 1. Do I need to wear hearing protection when I mow my lawn? 2. I already have a hearing loss because of my age. Do I still need to protect my hearing? 3. What about the hearing of my teenage grandchildren? Many common experiences such as lawnmower noise, listening to portable CD players while taking walks, and attending concerts—classical or otherwise—can subject the individual to potentially damaging levels of noise. However, it is not just the level [measured in decibels A-weighted (dBA)], but also the duration of the exposure that can cause hearing loss. For example, there is nothing wrong with going to a concert on Friday evening, as long as one does not mow the lawn on Saturday morning. Various models of hearing loss [see, for example, Ref. (2)] indicate that 85 dBA exposure for 40 hours per week is identical to an exposure of 88 dBA for only 20 hours per week, a 91 dBA exposure for only 10 hours per week, and so on. That is, a 3-dB increase effectively halves the exposure time required to cause damage. While a senior citizen may not be exposed to 85 dBA of noise or music for 40 hours per week, they may be exposed for 5 to 10 hours per week at a deceptively high level. Maximum weekly ‘‘dosages’’ can be obtained by five hours of exposure to a portable music player at a volume

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of 4 out of 10. For those who like to listen to ‘‘portable music’’ while they walk, enjoy boating, and mow their own lawn, hearing protection is advised for the lawn work. Does a previously existing hearing loss make a person more or less susceptible to further deterioration? Borg et al. (3), in reviewing a number of studies, concluded that just because a person already has a sensorineural hearing loss, they are no more and no less susceptible to future hearing loss from noise. The research indicates that it is just as important for a senior citizen to protect their hearing as it is for someone in their youth. The third question suggests that senior citizens have an increasingly important role to play in advising their grandchildren about the maintenance of healthy hearing. Young people may not listen to mom and dad, but increasingly our senior citizens are being looked upon as being cool!

HEARING PROTECTION Hearing protectors come in two forms—earplugs and earmuffs. As the name suggests, earplugs are inserted into the ear canal and earmuffs are worn over the ear and held to the head by a spring-loaded headband. Earplugs can be ‘‘one size fits all’’ or a custom fit where the individual has an impression taken of their ear. Other than very specialized industrial applications, hearing protection does not cover or surround the entire head. Subsequently, there is a theoretical upper limit on the attenuation provided by any hearing protector. Many studies have examined the basis for the maximum attenuation levels from hearing protection (4–8). These factors pertain not only to the thickness and density of the material used in hearing protectors, but also to the level of bone-conducted sound transmission. For example, Berger (8) noted that at 2000 Hz, the bone conduction limit for hearing protection was only 40 dB. That is, hearing protection attenuations of greater than 40 dB in the 2000 Hz region are artifacts of the measuring system. Above this 40 dB range, sound is conducted through the temporal bone directly to the cochlea, by passing the air conduction route. Figure 1 shows the bone conduction limits across frequency for hearing protector attenuation. Closely related to this is what is known as the occlusion effect. This is where there is an improvement of the lower frequency bone conduction thresholds upon occlusion of the ear canal. This results in the amplification of internal physiological noise (9). The essential characteristics of this phenomenon were first described by Josef Zwislocki in the 1950s (4,5). This occlusion effect is well known to hearing aid wearers. It typically results in an echo-like sensation being reported by hearing aid users. Extending the bore of the earplug into the inner or bony portion of the external ear canal can reduce the occlusion effect. This is one advantage of a custom earplug over a ‘‘one size fits all’’ type. The extent of the effect can be measured clinically in most audiology clinics. Another feature of hearing protection is that the majority attenuate higher frequency sounds more than lower frequency sounds. Figure 2 shows the frequencydependent characteristics of a typical industrial foam plug inserted deeply into the ear canal. If these plugs were not as deeply seated, low-frequency attenuations

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Figure 1 The maximum attenuations for bone conducted sound are shown, indicating that the maximum possible hearing protection attenuation in the 2000 Hz region is on the order of 40 dB. Maximum attenuations can exceed 40 dB at other frequencies however. Source: Adapted from Ref. 8.

of only 10–15 dB would be achieved. Two acoustic phenomena account for this frequency dependence: 1. Low-frequency sounds (i.e., long wavelengths) are acoustically ‘‘myopic’’ and do not see the obstruction well, while the higher-frequency (i.e., shorter wavelength) sounds acoustically ‘‘see’’ the obstruction. Thus, hearing protection will attenuate the higher-frequency consonants to a greater degree than the lower-frequency vowels and this creates ‘‘muffled’’ speech. 2. The human ear canal is a 25 mm (one inch) long tube that is closed at the tympanic membrane side and open at the other. It can be thought of as a quarter wavelength resonator with a frequency in the 2700 Hz region.

Figure 2 Typical attenuations for a deeply seated industrial foam earplug with maximum attenuations being less than 40 dB. A more shallow ear insertion would reduce the attenuation by 10–15 dB, especially for the lower frequencies.

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Due to this configuration, the ear canal naturally amplifies consonants in the 2700 Hz region (the top octave of a piano keyboard) by about 17–20 dB. Whenever the ear canal is occluded (with a hearing aid or an earplug), this naturally occurring resonance is lost. Plugging the ear canal is acoustically comparable to a musician inserting the hand into the bell of the French horn. With earplugs, high-frequency (insertion) loss is caused by the loss of the 2700 Hz resonance. The net effect from both of these physical phenomena is that high-frequency consonant sounds are attenuated more than lower-frequency ones. In contrast, earmuffs do not modulate the natural ear resonance in the 2700 Hz region. This results in less relative high-frequency attenuation than if earplugs were used. Earmuffs do, however, yield greater attenuations in the 500–1000 Hz region. As an additional side effect, the level of the occlusion effect will be less with large volume earmuffs than with many earplug fittings. Even though many earmuffs provide a ‘‘more uniform’’ attenuation pattern than earplugs, one cannot state definitively that the use of earmuffs yield improved speech intelligibility in noise over that of the use of earplugs, since hearing loss configuration and large subject variability are confounding factors (10).

ACOUSTICALLY (AND ELECTRONICALLY) TUNED HEARING PROTECTION Many people do not like to wear hearing protection. This is understandable given the nonuniform attenuation characteristics, the occlusion effect, and reduced speech intelligibility in noise. In order to resolve this problem, Killion et al. (11) devised a custom earplug with approximately 15 dB of attenuation over a wide range of frequencies. The earplug, named the ER-15TM (manufactured by Etymotic Research, Inc.), has become widely accepted by musicians and music lovers, as well as some industrial workers who work in relatively quiet environments (<100 dBA). There is a noncustom form of this earplug called the ER-20. As the name suggests, this plug attenuates all sounds by about 20 dB (12). Figure 3 shows the attenuation characteristics of the ER-15 earplug and an industrial-type foam plug (from Fig. 2) for comparison purposes. Bilsom (another manufacturer of hearing protection) has come out with the NSTTM earplug. NST stands for ‘‘natural sound technology’’ and is a noncustom earplug giving a relatively flat attenuation characteristic. Three benefits of a ‘‘flat attenuation’’ earplug are: 1. Hearing sounds (especially music) the way they were intended. 2. An improved ability to communicate in a noisy environment (over conventional hearing protection). 3. Improved localization for the direction of sound. Although it can be argued that this third point has relatively little importance for people not working in a noisy environment, the first two will certainly improve one’s enjoyment of music. It is a fallacy that more sound attenuation is better. As long as the earplug or earmuff is adequate, meaning that there is enough attenuation for the noise/music

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Figure 3 The attenuation characteristic of the custom ER-15 uniform attenuator earplug and for comparison purposes, the attenuation of a deeply seated industrial strength foam plug (Fig. 2).

environment—more is not better. More than the required attenuation can reduce speech intelligibility and reduce the overall ability to communicate. For many retired adults, their children, and grandchildren, 15–20 dB of attenuation is quite sufficient, while listening to music or attending a concert. Industrial strength (up to 40 dB) attenuation is not typically required for this type of recreational activity. Hearing protection based on electronic characteristics rather than acoustic ones have recently been introduced. The first, active noise reduction (first reported on in 1933) is based on the principle that two identical signals when out of phase and added together will cancel each other out. While it is difficult to assess a quickly varying signal and generate another with the identical spectrum but 180 out of phase, it is a relatively easy task for steady-state signals, especially if they are low frequency (i.e., long wavelength). Active noise reduction hearing protection has not gained wide acceptance because of high cost, frequency limitation, and until recently a limitation to relatively steady-state signals. Recently, it has been gaining acceptance for certain uses such as long airplane flights. These earmuffs can cut out the constant drone of the airplane cabin. They can also be connected to a portable music player. The earmuffs, in cutting out some background noise, allow the listener to hear music at lower volumes, thus reducing the overall risk to hearing. By cutting out some lower frequency environmental noise, stress is also reduced. In contrast, active sound transmission hearing protection has had its major use in hunting where optimal hearing of the movement of the prey has to be coupled with optimal hearing protection. A modified hearing aid circuit is typically utilized that allows for amplification at low input levels but attenuation or clipping at higher levels (such as the blast from a gun). An external monitoring microphone is used to assess the environmental noise/music level, and the amplification stages are reduced or disabled completely above a certain preselected level.

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There are some reports in the literature regarding the use of compression or level-dependent hearing aid circuits such as the K-AMP, as well as the use of hearing aids that have been turned off (13,14). These have been shown to be quite useful, even for those with mild hearing losses, as long as there is no vent. Many hearing aid manufacturers are offering a relatively new device—a combination of hearing aid and custom active hearing protector. In the hearing aid mode, such a device provides up to 40 dB of gain, but in the hearing protection mode becomes level dependent with peak gains of up to 15 dB. When the device is physically turned off, the shell provides attenuations of 25 dB in the lower frequencies and up to 35–40 dB in the higher frequencies. Many senior citizens wear custom made hearing aids. Increasingly, these hearing aids use compression circuits that serve to not only amplify the softer sounds of speech but also attenuate or decrease the level of the louder environmental sounds. In this sense, many modern hearing aids are an example of electronic hearing protection. Not all hearing aids use the same circuitry, so it is important for the individual to contact their hearing health care professional to determine if their current amplification can be used as a hearing protector in noisy environments.

RATING HEARING PROTECTION Many commercially available forms of hearing protection come with a noise reduction rating number on the package. This number can be misleading because a higher number is not necessarily better. Several single number rating schemes are in use throughout the world. The most commonly used scheme in the United States is the noise reduction rating (NRR). Most parts of Europe use an octave band method as well as the International Organization for Standardization (ISO) recommended single number rating (Berger, personal communication, 1995). Most provinces in Canada use the ABC scheme where hearing protectors are categorized according to a standard (Z94.2-94) into Class A, B, or C depending on their octave band attenuation values and the measured Leq—a time-weighted average (15). NRR has been in widespread use since 1979. However, Preves and Pehringer (16) have pointed out that the NRR calculation makes some simplifying assumptions and requires the use of various correction factors. The validity of these assumptions has led to some criticism of the NRR technique. For example, the National Institute for Occupational Safety and Health (NIOSH) felt that there should be a correction factor of 3 dB in the calculation of the NRR for reasons pertaining to spectral uncertainties. As this figure would be subtracted from the calculation, the NRR would be a worst-case scenario. NRR for each hearing protector is a measure based on the results of a number of subjects. Subsequently, a two standard deviation pad is also included as ‘‘a statistical adjustment so that the mean values are modified to reflect what some larger proportion of the population will actually achieve’’ (Berger, personal communication, 1995). This two standard deviation range covers approximately 95% of the population. Although simplifying measures such as the NRR have made hearing protector characterization easier for the laboratories, the NRR tends to be affected by many artifacts when used for a different purpose than originally intended. It is, unfortunately, a widely held view that the higher the NRR, the better the hearing protector.

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Depending on the noise or music level, the spectral shape, and the individual’s communication or musical requirements, this is certainly not the case. STRATEGIES FOR GRANDCHILDREN TO PROTECT THEIR HEARING This section can be given as a handout to patients who have younger children or grandchildren. It is therefore written with the lay public in mind. 1. Humming: Most people with healthy ears have a natural sound attenuator in their middle ears called the stapedial reflex. This neurological reflex causes the stapedial muscle to contract upon loud sounds, thereby stiffening the middle ear bones or ossicles. This has the effect of reducing the transmission ability of the middle ear with the result that loud sounds do not get to the inner ear as effectively. All mammals have this reflex to varying degrees. From an evolutionary perspective, we have such a reflex to protect ourselves from our own voices. Depending on the study, this reflex has been shown to provide anywhere between 3 and 15 dB of hearing protection. Even if it is only 3 dB, this still cuts the damaging noise or music exposure in half so that a person can listen twice as long before the same damage occurs. A strategy to take advantage of this would be to elicit this natural reflex before a loud sounds such as a cymbal crash or when walking past a construction site. The stapedial reflex can be elicited simply by humming. This ‘‘fools’’ your ears into thinking that there is loud noise. Sustaining this hum throughout the loud sound will provide substantial hearing protection. 2. Hearing protection that attenuates sounds yet lets guitars still sound like guitars, drums like drums, and horns like horns is available. Such protection is widely available through most local hearing health care professionals. Many famous musicians and sound engineers wear this type of hearing protection and indeed forget that they are wearing anything in their ears. 3. Hearing loss is much like a dose of radiation. Small doses for short periods of time are not damaging, but even if relatively quiet noise or music exposure is heard for an extended period of time, hearing loss can ensue. As a rule of thumb, it takes the human ear about 16–18 hours to recover from loud noise. For the first 16–18 hours after a concert you may be suffering from a temporary hearing loss—usually noticed as a feeling of fullness or dullness, as if a piece of cotton is in your ear. Additional exposures within this critical 16–18-hour period would add significantly to the dose. However, additional doses of noise or music exposure separated by at least 16–18 hours do not contribute as significantly to the dose. Go ahead and enjoy the concert on Friday night, but do not mow your lawn until Sunday or better yet, get someone else to do it for you! ACKNOWLEDGMENTS Some of this information was derived from A. Behar, M. Chasin, and M. Cheesman, Noise Control—A Primer, San Diego: Singular Publishing Group, 2000.

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REFERENCES 1. Chasin M. Musicians and the prevention of hearing loss. San Diego: Singular Publishing Group, 1996. 2. International Organization for Standardization (ISO). Acoustics—determination of occupational noise exposure and estimation of noise-induced hearing impairment. 2nd ed. International Standard ISO 1999. Geneva, Switzerland: Author, 1999. 3. Borg E, Canlon B, Engstrom B. Noise induced hearing loss: literature review and experiments in rabbits. Scand Audiol 1995; 24(suppl):40. 4. Zwislocki J. Acoustic attenuation between the ears. J Acoust Soc Am 1953; 25:752–759. 5. Zwislocki J. In search of the bone-conducted threshold in a sound field. J Acoust Soc Am 1957; 29:795–804. 6. Shaw EAG, Theissen GJ. Improved cushion for ear defenders. J Acoust Soc Am 1958; 30:24–36. 7. Shaw EAG, Theissen GJ. Acoustics of circumaural earphones. J Acoust Soc Am 1962; 34:1233–1243. 8. Berger EH. Methods for measuring the attenuation of hearing protection devices. J Acoust Soc Am 1986; 79:1655–1687. 9. Berger EH, Kerivan JE. Influence of physiological noise and the occlusion effect on the measurement of real-ear attenuation at threshold. J Acoust Soc Am 1983; 74:81–94. 10. Abel SM, Alberti PA, Haythornwaite C, Riko K. Speech intelligibility in noise with and without ear protectors. In: Alberti PW, ed. Personal Hearing Protection in Industry. New York: Raven Press, 1982:371–386. 11. Killion MC, DeVilbiss E, Stewart J. An earplug with uniform 15-dB attenuation. Hear J 1988; 41(5):14–16. 12. Killion MC, Stewart J, Falco R, Berger EH. Improved audibility earplug. U.S. Patent 5,113,967, 1992. 13. Killion MC. The parvum bonum, plus melius fallacy in earplug selection. In: Beilin L, Jensen GR, eds. Recent Developments in Hearing Aid Technology (15th Danavox Symposium). Kolding, Denmark: Scanticon, 1993:415–433. 14. He´tu R, Tran Quoc H, Tougas Y. Can an inactivated hearing aid act as a hearing protector? Canad Acoust 1992; 20(3):35–36. 15. Behar A, Desormeaux J. NRR, ABC, OR. Canad Acoust 1994; 22(1):27–30. 16. Preves DA, Pehringer JL. Calculating individual NRRs in situ using subminiature probe microphones. Hear Instrum 1983; 33(3):10–14.

9 Multisensory Impairment in Older Adults: Evaluation and Intervention Susan L. Whitney Departments of Physical Therapy and Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

Laura O. Morris Balance and Vestibular Program, Eye and Ear Institute, Centers for Rehab Services, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION Multisensory deprivation disequilibrium is a condition that most commonly develops in the aging population. It occurs due to degradation of the three primary sensory systems used in balance: somatosensory, vestibular, and visual systems. These systems provide sensory information throughout the central nervous system regarding body position. The information is then coordinated and compared to memory of previous experiences and spatial maps. Areas of the cortex then develop a coordinated response from the neuromusculoskeletal system in order to maintain upright balance and perform such diverse activities as catching a ball or locomotion. This chapter addresses the clinical examination of the three primary sensory systems: somatosensory, vestibular, and visual. No individual system provides all the sensory information required to determine position in space. Each system contributes its own unique and important information about body position and movement. As the normal older adult ages, some degradation of all three systems occur. In the healthy, active, aging adult, these changes are minimal and are inapparent. Decrement in the ability of the individual to perform balance-sensitive physical activities such as snow skiing or tennis is present, but inapparent. Peak performance may not be at the same level as someone younger; hence, older athletes characteristically compete within their own age group. A sedentary lifestyle with reduced levels of physical activity and stimulation can lead to decreased functional abilities purely due to deconditioning. Social factors such as a loss of a spouse, changing living environments, or retirement can lead to further inactivity, degrading a person’s ability to maintain balance. The risk for developing diseases that affect the sensory systems is greater in older adults. In isolation, a single impairment may not appear to have a significant 109

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effect on balance. However, defects in multiple systems can result in a devastating effect on an individual’s ability to function. The relationship between incremental increase in sensory system decrement and functional loss is not linear as the systems are interconnected and interdependent. When multiple systems are involved, the term multisensory impairment is used. Older adults experiencing multisensory impairment usually complain of imbalance, unsteadiness, dizziness, lightheadedness, and/or fear of falling when standing or walking. The symptoms usually subside with sitting or holding onto a stationary support. This improvement is due not to physical support, but due to increased proprioceptive input as to body position. A cane functions as much as a ‘‘feeler’’ as an extra supporting appendage. Typically, these symptoms do not develop acutely but gradually over a number of years. Patients may report a gradual onset of imbalance that has led to a decline in their ability to manage in dark environments or walk on uneven surfaces in their homes or community. Fear of falling may result in self-imposed limitations of activity. This reduction in activity further compromises function due to deconditioning and leads to complaints of fatigue and unsteadiness. Many older persons report feeling ‘‘dizzy’’ when in actuality they feel a sense of imbalance. Terms such as lightheadedness may also be used for feeling unsteady or for presyncope. Those experiencing multisensory impairment may rarely report a sense of spinning or vertigo, which is a hallmark sign of vestibular impairment, but this is an uncommon description. Dizziness may also be due to systemic factors such as cardiovascular or metabolic abnormalities. It is often difficult to get an accurate description of symptoms, but it is worth the effort in order to accurately assess the etiology of symptoms. The prevalence of dizziness in the elderly population is significant. Aggarwal et al. (1) reported an average prevalence of 9.6% in those over the age of 65, with those over the age of 85 reporting dizziness 18.4% of the time. The population worldwide is becoming older at a higher rate, which increases the importance of addressing multisensory impairment in older persons.

NORMAL AGE-RELATED CHANGES Changes in sensory systems have been well documented in the literature. Baloh et al. (2) documented a decrease in gain of the vestibulo-ocular reflex in a longitudinal study of normal older adults. A loss of neurons in the vestibular nucleus between 40 and 90 years of age, occurring at a rate of approximately 3% per decade, has also been reported (3). Rauch et al. (4) documented a loss of hair cells, thereby decreasing the sensitivity of the peripheral system to high-frequency head movement that is often required for many activities of daily living. There are reductions in the gain of smooth pursuit and increases in saccade latencies with age. Somatosensory changes in the older adult lead to a two- to tenfold decrease in the vibration threshold, indicating a reduced ability to feel the quality of contact between themselves and the surface below (5,6). A decrease in muscle spindle activity, sensitivity of skin receptors, and joint receptor activity also negatively influence postural control. Brocklehurst et al. (7) and Lord et al. (8) found that this age-related loss in somatosensation led to impaired control of postural sway and higher risk of falling. The visual system degrades with age as well. Common age-related changes include a decrease in visual acuity, depth perception, and contrast sensitivity. The visual field narrows, reducing peripheral visual input. Behaviorally, these changes adversely affect the older adult’s ability to accurately perceive their environment, including changes in

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surface conditions. Difficulty in avoiding obstacles, negotiating curbs and stairs, and efficiently moving in low light conditions may result. An increased risk for falling due to visual impairments is well documented and outlined later in the chapter.

PATHOLOGICAL SENSORY CHANGES IN THE OLDER ADULT Although many older adults age successfully without documented pathology, the risk for developing many disease processes is higher. The labyrinthine system is vulnerable to vascular changes due to atherosclerosis, caused by cardiac disease or diabetes mellitus. Medications that impair balance may exert a greater effect on the older adult. The somatosensory system can be affected by peripheral neuropathy. According to Richardson and Ashton-Miller (9), the prevalence of peripheral neuropathy in developed countries may be as high as 20%. Diabetes mellitus accounts for many of these cases, but as many as 10% of adults have peripheral neuropathy from other causes. Richardson et al. (10,11) also found that older adults with identified peripheral neuropathy on electromyogram (EMG) had an increased risk of falling by a factor of 20 when compared to age- and sex-matched adults with normal peripheral nerves. Changes to the visual system due to disease processes can also affect the older adult. Diabetes mellitus, multiple sclerosis, and myasthenia gravis can cause visual changes. Cataracts reduce visual acuity, although lens implants can now significantly reduce this visual deficit. Macular degeneration reduces foveal vision, while essentially maintaining the peripheral visual field. In contrast, glaucoma reduces peripheral vision, creating ‘‘tunnel vision.’’ All of these diseases significantly impact an elderly adult from being able to accurately perceive their environment. In addition to peripheral changes due to pathology, changes to the central nervous system (CNS) and its ability to integrate information are also more common in the older adult. Stroke and vascular changes in the CNS can affect integration and processing of information. Kerber et al. (12) found that higher ventricular-brain ratios, more frontal lobe atrophy, and more subcortical white matter lesions were found on magnetic resonance images (MRIs) in older adults with disequilibrium than with matched-age controls.

SOMATOSENSORY SCREENING Several aspects of somatosensory function should be screened in order to determine the function of this system. A thorough screening should not take more than a few minutes and will be essentially painless for the patient. Because the primary focus is on balance function, these tests should be performed on the lower extremities, with the shoes and socks removed. Figure 1 illustrates a somatosensory screening test. As will be outlined in more detail later in the chapter, the use of a pain and sensation diagram may be helpful in gaining the patient’s perception of sensory loss. Often the patient is unaware of the sensation loss or the extent of the impairment. Vibration Sense Vibration sense is tested by using a tuning fork. The vibrating fork is placed on a bony process, usually the first metatarsal head, the lateral mallelous, and/or the

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Figure 1 Proprioception should be tested by holding the lateral borders of the appendage and moving it up or down. The patient is asked to report whether their toe is ‘‘up’’ or ‘‘down’’ relative to the ground. Deficits in proprioception are never a normal finding in older adults.

fibular head or patella. The patient can be asked to vocalize the exact moment when the vibration stops, and the tester can stop the vibration with their fingers while maintaining contact of the fork with the skin. Another method, less objective, is to have the patient compare the tuning fork vibration on one extremity to the other. The difficulty with this comparison is that the vibration provided to the patient will vary depending on how hard one taps the tuning fork. Proprioception In order to test proprioception, one must hold both a proximal joint and the distal limb by its bony processes and move the distal limb up and down in small excursions (Fig. 1). It is important that one not touch too much of the toe or you will provide additional sensory input, making it easier for the subject to be accurate with the testing. The patient is asked to give the direction that the limb is moving while the eyes are closed. It is important to remember that the proprioceptive system is very sensitive and can sense movement in as little as 0.3 , so movement should be in very small excursions (9). Another gross test of proprioception, called placing, is to hold the limb in a similar manner and ask the patient to place the opposite limb in exactly the same position. Gross dysfunction can be ascertained quickly with this test. If deficits are noted, a neurologist should be contacted. Cutaneous Sensation Light touch is tested with the patient sitting with eyes closed, and the tester lightly touching the skin. Moving touch requires the patient to verbalize the direction that the tester moves on the limb. Taking two sharp objects, such as both ends of a protractor, tests two-point discrimination while randomly touching one or two points to the patient’s skin. The patient must determine how many points are touching. The points can be varied in distance from one another to determine sensitivity. In sharp/dull testing, the tester randomly places a sharp or a dull object on the patient’s skin, and the patient is asked to identify the object as either sharp or dull. All peripheral nerve and dermatome areas should be included in the testing.

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Hot and Cold Sensation Hot and cold sensation is impaired in many disease processes, and the patient may not be aware of the deficit. Loss or impaired hot/cold sensation can lead to significant safety risk. One way to test hot and cold is to use a metal object that has been heated or cooled, and place it on the patient’s skin. Test tubes or similar containers of hot and cold water can also be used. As many patients have sensitive skin, one must ensure that the temperature is not too extreme. Laboratory Tests of Sensory Loss A nerve conduction velocity test can be performed by a physician or physical therapist and is used to assess sensory loss, tingling, pain, muscle cramping, or unexplained weakness distally. Typically, the test does not cause much pain as an electric shock is sent through the nerve. The test provides information about the speed of the nerve transmission. The nerve conduction velocity is most often accompanied by the more painful needle EMG test. A fine wire needle is inserted into the muscle to determine the speed of nerve transmission and also the examiner will ‘‘listen’’ to the sound of the muscle to make the diagnosis. The test takes approximately 25 minutes to 1.5 hours to perform.

VISUAL SCREENING Directional E Test Screening a person’s visual acuity is important and can be performed in the physician’s office. The easiest way to quickly assess vision is to use a directional E eye chart to determine the patient’s acuity. Several studies have related falls to impairments in vision (13–16). Abdelhafiz and Austin (17) suggest that visual impairments are related to hip fracture in older persons. In addition to screening for visual acuity, depth perception and contrast sensitivity can be screened as both additional visual characteristics have been related to falls in older persons. Although the directional E test is the easiest to perform, testing contrast sensitivity will provide the clinician with greater insight into the patient’s fall risk. Contrast Sensitivity An effective screening tool that has been directly related to falls is impaired contrast sensitivity (14). When testing contrast sensitivity, one has the patient read the letters until they can no longer see any (Fig. 2). The letters at the top of the chart are dark and then gradually become lighter. Poor contrast sensitivity has been related to falls risk. Having sharp contrast at the edge of objects such as curbs and the edge of steps can aid persons with impaired contrast sensitivity. Macular Degeneration Jack et al. (18) suggest that low vision is related to falling in older adults admitted to the hospital. A through eye history will help the physician better understand their patient’s balance deficits when they present with multisensory disequilibrium. Referral to an ophthalmologist who specializes in low vision might be a consideration.

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Figure 2 Testing for contrast sensitivity. A person stands in front of the chart and attempts to read as many letters as possible on the wall.

Cataracts Brannan et al. (16) studied fall rates before and after cataract surgery. They demonstrated that after cataract surgery, person’s fall rates decreased from 37% to 19% six months following surgery. As part of the history, it is important to determine if the person presently has cataracts.

Visual Field Testing In order to determine if the patient has normal visual fields, the physician can sit in front of the patient or stand behind them. One should ask patients to look straight ahead and to tell you when they see your finger appear in their periphery. The physician is to move a finger from behind the patient’s head towards the front on both the right and left sides and from over the top of their head. Looking for right/left symmetry in responses is important. Deficits in visual field testing could indicate either a peripheral or central disorder and if identified, would need further investigation to determine the location of the deficit.

Multifocal Lenses Determining if the patient has multifocal lenses is important. Lord et al. (19) suggest that multifocal lenses impair both edge-contrast sensitivity and depth perception. These factors may predispose a person to greater risk of falling, especially on stairs. Asking about what type of lenses they have and when their last vision screen was is an important component of a through assessment of an older adult. If possible, having separate reading and distance lenses can help to solve the problem.

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Cranial Nerves 3, 4, and 6 Assessing the motor performance of cranial nerves 3, 4, and 6 takes only a few seconds but can provide a wealth of information. The easiest method is to ask the patient to follow an H that you draw with your finger in front of the patient’s face. When the eyes are following your finger, you are assessing all of the extraocular eye movements. For example, right and left horizontal eye movements assess the integrity of the lateral and medial rectus. If a deficit is identified, the patient should be referred to either an opthalmologist or a neurologist.

VESTIBULAR SCREENING FOR OLDER PEOPLE There are several clinical tools that can be used to attempt to determine if the patient has a peripheral vestibular disorder. None are foolproof and the best way to test for vestibular dysfunction is through the use of a full vestibular test battery including calorics, an oculomotor screen, rotational chair testing, and computerized dynamic posturography. Sometimes circumstances do not permit the testing. The sensitivity and specificity of various clinical tools will be described. Dix-Hallpike Test The Dix-Hallpike test is crucial to assess in older adults as many patients have benign paroxysmal positional vertigo (BPPV), but it has not been diagnosed (20). It is suggested that the person start in long sitting with the head rotated 45 to either the right or the left. Then the person is brought to the head hanging position (approximately 30 over the edge of the bed). Excessive extension should be avoided in older persons as it might compromise their vertebrobasilar circulation. The speed of the Dix-Hallpike may need to be modified in older persons. If done too quickly, the person might experience a neck injury. With persons with a positive history of BPPV, sometimes a repeat Dix-Hallpike may be positive whereas the first maneuver was negative. If positive for a posterior canal disorder (the most common), the patient will exhibit a torsional upbeating nystagmus. The characteristic nystagmus is best visualized with Frenzel lenses or an infrared goggle system. One must be careful to not perform the Dix-Hallpike test with persons with neck problems (cervical stenosis, cervical radiculopathies, significant vertebral artery disease, disc disease) and with persons with additional spinal deformities (Pagets disease, spinal cord injuries, low back dysfunction, and ankylosing spondylitis), depending on the severity of the disease. Persons with a history on examination of transient ischemic attacks and rheumatoid arthritis should also be carefully assessed. If there is a concern about performing the Dix-Hallpike test, one might consider using a tilt table to perform the maneuver. The head position changes in relation to gravity while on the tilt table but you will not be changing or stressing the neck and back of the older adult. Older adults feel more comfortable performing the test with their legs flexed in order to avoid low back pain. Having another person present can help to decrease their anxiety of having the test performed, as there is fear associated with placing the patient in the provocative position. In addition, one can have the older adult with restricted neck motion move their entire body to the side if they do not have adequate neck range of motion prior to performing the Dix-Hallpike maneuver. If you decide that it is too difficult to

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assess the person in the office and send the patient to a physical therapist for the assessment and treatment of the BPPV, it is imperative that you provide details of the patient’s laboratory testing, your concerns about doing the Dix-Hallpike, and your suspicion of BPPV. They will be able to manage the patient more successfully with greater knowledge of the patient’s medical history and with lower risk of harm to the patient. The Head Thrust The head thrust test is a high acceleration movement of the head typically performed in the plane of the horizontal canal (21,22). It can also be performed in the other two planes of the semicircular canals. The patient tries to maintain fixation on a distant object during the test and one attempts to determine if the patient produces a catch up saccade. The second law of Ewald (Ewald, 1892, in Ref. 23) suggests that an asymmetric response occurs during the high-velocity movement. It must be performed passively, be unpredictable, and be of high acceleration to see the compensatory eye movement (22). The sensitivity of the test has been reported to be 54% and specificity was reported to be 100% (24). Gaze Holding It is important to determine if a patient can focus on an object without nystagmus. The patient is asked to focus on a nonmoving object. One can best visualize the nystagmus with an ophthalmoscope, as it is difficult with just one’s vision to assess if there is movement. One should focus on the fundus to determine if there is movement. If there is movement, it could indicate that the person has an acute peripheral disorder or a brain disorder. Questionnaires In an older adult, in order to assess somatosensory impairments, one should have the patient complete a pain diagram (Fig. 3). On the diagram, which depicts the ventral and dorsal aspect of the body, the patient has an opportunity to describe any pain or sensory loss that they might be experiencing. The pain scale can be quantified and a comparison can be made in future visits to the clinic. Another helpful paper and pencil tool that can provide additional information to the physician is the Activities-Specific Balance Confidence (ABC) Scale (25–27). It is a 16-item questionnaire that was developed to assess balance confidence in older persons that is rated on a 10-point scale from 0 to 100. Scores are added and then divided by 1600 for a percent score. The ABC has been used in persons with vestibular disorders. In older adults, scores of less than 80 indicate functional impairments and scores of less than 50 indicate that the person is often housebound (28). The ABC could be used as a screening tool to determine the patient’s perceived functional impairment (29). The staff can supply and score the questionnaire prior to the patient’s physician interview. A third questionnaire particularly helpful to an otolaryngologist is the Dizziness Handicap Inventory (DHI) (30). The DHI consists of 25 items that assist the physician in determining the patient’s extent of perceived dizziness. With DHI scores of >60, people had an increased chance of reporting a fall (Whitney et al., in press).

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Figure 3 A pain diagram that can be used to determine if the patient has sensory loss or impairment.

Quick Screening Techniques to Assess Fall Risk One of the easiest methods to assess fall risk and also help in the diagnosis of a vestibular disorder is to have the older adults stand on a piece of high-density temper foam (T-foam). Weber and Cass (31) suggested that the ability to stand on the T-foam correlated to computerized dynamic posturography. They reported a sensitivity of 95% and a specificity of 90% in persons with vestibular dysfunction on conditions 4 and 5 (standing on foam, eyes open and closed). The foam pad must be placed on a nonslip surface and the patient must be carefully guarded to ensure that they do not fall. If the person falls off the foam with eyes open, it suggests that they have difficulty walking on uneven surfaces, even with good light. When falling off the T-foam with the eyes closed, it may suggest a vestibular disorder. These persons will report that they have difficulty walking in the dark. One must closely guard the patient as they attempt to stand on the high-density T-foam, as it is not uncommon for them to fall off the foam.

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An easy way to assess overall strength is to ask the person to move from sit to stand without using their arms. If they have difficulty with this task, they are at risk for falling (32). The Romberg test can help to provide an assessment of overall dorsal column function. It was designed as a test of lower extremity sensation. Persons with diabetes are unable to maintain the Romberg position and many community-living older adults also have difficulty performing the Romberg test. Not all older adults with vestibular dysfunction fall during the Romberg test. Guard the person closely, as it is not uncommon for the person to fall. When assessing an older adult, it is recommended that all three tests be performed. If they fall during the Romberg test, it may or may not indicate significant pathology in the older person. A positive Romberg is seen in community-living older adults. If the patient falls during standing on the foam pad and/or cannot rise from the chair, a referral to physical therapy may be indicated.

INTERVENTION FOR MULTISENSORY DISEQUILIBRIUM Once an assessment of sensory systems is complete and impairments identified, the next step involves appropriate medical and rehabilitative intervention. A review of medications is always advised in order to identify possible adverse effects. A careful review of the history may reveal an underlying etiology, as outlined in Table 1. If any of the sensory impairments can be treated medically, the appropriate physician should address this. Table 2 lists disorders and diseases that can cause sensory or gait dysfunction in older adults. Rehabilitation for multisensory disequilibrium has been found to be effective in managing the balance deficit and functional impairment. A systematic approach of assessing both sensory and motor deficits, functional impairment, and disability is followed by treatment that addresses education and improving function. Fall risk reduction education is an integral part of the therapy process, as described in Chapter 14, ‘‘Balance Rehabilitation.’’ If a sensory system has a partial or temporary impairment (visual, vestibular, or somatosensory), the therapist attempts to improve function of the impaired system by increasing the weighting or use of that system while decreasing the use of the other two sensory systems. For somatosensation, the therapist will encourage eyes closed activities on a firm surface in order to decrease the use of visual feedback. Also, Maki et al. (33) demonstrated that patients with cutaneous impairment improved in postural reactions with a raised boundary on the plantar surface of their feet. Older adults also demonstrated less postural sway when they wore vibrating insoles compared to young persons (34). Physical therapists may be able to provide avenues for patients to improve somatosensation and thereby improve overall stability. If damage to a sensory system is permanent or progressive, in the case of complete bilateral vestibular loss or severe visual impairment, rehabilitation focuses on compensation by improving the use of the ‘‘better’’ functioning sensory systems (35). In some cases, the best avenue for intervention is to improve strength and motor function, and to teach compensation for the sensory impairment(s). The use of a straight cane is a good compensatory tool for impaired visual and somatosensory systems (36). The upper extremity can assist in providing extra somatosensory feedback, while the cane helps in assessing depth and uneven surfaces that the visual system may not be able to perceive (36,37). Reversing the effects of deconditioning is essential in this population, as the lack

Visual impairment: cataracts, glaucoma, multifocal lenses, visual field deficit; stroke, head trauma Multisensory disequilibrium

Assess sensation, vision, and balance

Visual screen, refer to ophthalmologist

Treat conservatively for Meinere’s disease depending on their risk of fracture and send to a neurologist Needle EMG, vibration, and distal sensory testing

Abbreviations: EMG, electromyogram; MRI, magentic resonance imaging; BPPV, benign paroxysmal positional vertigo.

I am losing confidence; I feel that I need a cane, I am afraid to leave the house.

I feel like my feet have lead in them—they are heavy. I keep bumping into things and I can’t see as well. I don’t see people coming up next to me.

I fall down but do not pass out.

I faint sometimes and have fallen.

Autonomic dysfunction, transient ischemic attack Attack of Tumarkins or a true drop attack Peripheral neuropathy, diabetes

Stroke, head injury, chronic subdural hematoma BPPV

Dix-Hallpike test and either treat or refer to a physical therapist Refer to a neurologist and/or cardiologist

MRI

Parkinson’s disease or a basal ganglia disorder

I am slowing down and falling. I lose my balance when I turn while walking. It is hard to roll over in bed. I lose my balance. Sometimes I see double.

I feel like I spin when I roll over in bed.

Supine, sitting, and standing BP (check for a drop in diastolic pressure of 10 mmmHg or more) Dix-Hallpike test Check for bruits Possible referral to a cardiologist Refer to a neurologist

Orthostatic hypotension or BPPV

I get dizzy when I get out of bed or when I go from sitting to standing.

Needle EMG Refer to a physical therapist

Tests to perform

Orthostatic tremor Severe muscle weakness

Possible diagnosis

I can’t stand for a long time. If I stand for longer than a few minutes, my legs start to shake.

Patient comments

Table 1 What Older Adults Complain of, Possible Working Diagnoses, and Tests to Perform to Confirm the Diagnosis

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Table 2 Disorders and Diseases That Can Cause Sensory Loss or Gait Disorders in Older Adults Brain disorders Basal ganglia disorders (Parkinson’s disease) Cerebellar degeneration Chronic subdural hematoma Dementia Depression Head trauma Normal pressure hydrocephalus Orthostatic tremor Stroke Transient ischemic attacks Spinal disorders Cervical myelopathy Mononeuropathy Peripheral neuropathy Spinal stenosis Spinal tumors Systemic disorders Alcoholism Diabetes Mellitus Neuropathy (HIV, Lyme disease) Vascular disorders Baroreceptor dysfunction Postural hypotension Visual disorders Macular degeneration Medication Medication-induced Status post organ transplant Anterior horn cell disorder Amytrophic lateral sclerosis Musculoskeletal Foot deformities Weakness

of balance leads to less physical activity, which only exacerbates the deficits. By providing a safe environment to exercise in a rehabilitation setting, as well as sound recommendations for activities at home, patients can increase their activity level safely with greater confidence (38,39). Efficacy of such rehabilitation has been documented by Rose and Clark and Shumway-Cook et al. (39–44) in which multiple and specific dimensions of the postural control system demonstrated significant improvements in balance and mobility. In summary, multisensory disequilibrium is a disorder that affects a significant number of older adults. It results in an overall sense of imbalance and instability while in an upright position. It involves degradation of the sensory systems responsible for balance function, both due to normal aging and pathological disease processes. The most effective way to manage the disorder is by a thorough assessment of the somatosensory, visual, and vestibular systems, and address intervention and rehabilitation according to the deficits identified. Improvement in overall

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functional mobility is sometimes possible with targeted intervention as long as appropriate causative factors are determined. REFERENCES 1. Aggarwal NT, Bennett DA, et al. The prevalence of dizziness and its association with functional disability in a biracial community population. J Gerontol A Biol Sci Med Sci 2000; 55(5):M288–292. 2. Baloh RW, Enrietto J, et al. Age-related changes in vestibular function: a longitudinal study. Ann NY Acad Sci 2001; 942:210–219. 3. Lopez I, Honrubia V, et al. Aging and the human vestibular nucleus. J Vestib Res 1997; 7(1):77–85. 4. Rauch SD, Velazquez-Villasenor L, et al. Decreasing hair cell counts in aging humans. Ann NY Acad Sci 2001; 942:220–227. 5. Perret E, Regli F. Age and the perceptual threshold for vibratory stimuli. Eur Neurol 1970; 4(2):65–76. 6. Bergin PS, Bronstein AM, et al. Body sway and vibration perception thresholds in normal aging and in patients with polyneuropathy. J Neurol Neurosurg Psychiatry 1995; 58(3):335–340. 7. Brocklehurst JC, Robertson D, et al. Clinical correlates of sway in old age—sensory modalities. Age Ageing 1982; 11(1):1–10. 8. Lord SR, Ward JA, et al. Physiological factors associated with falls in older communitydwelling women. J Am Geriatr Soc 1994; 42(10):1110–1117. 9. Richardson JK, Ashton-Miller JA. Peripheral neuropathy: an often-overlooked cause of falls in the elderly. Postgrad Med 1996; 99(6):161–172. 10. Richardson JK, Ching C, et al. The relationship between electromyographically documented peripheral neuropathy and falls. J Am Geriatr Soc 1992; 40(10):1008–1012. 11. Richardson JK, Hurvitz EA. Peripheral neuropathy: a true risk factor for falls. J Gerontol A Biol Sci Med Sci 1995; 50(4):M211–215. 12. Kerber KA, Enrietto JA, et al. Disequilibrium in older people—a prospective study. Neurology 1998; 51(2):574–580. 13. Lord SR, Menz HB. Visual contributions to postural stability in older adults. Gerontology 2000; 46(6):306–310. 14. Lord SR, Dayhew J. Visual risk factors for falls in older people. J Am Geriatr Soc 2001; 49(5):508–515. 15. Tinetti ME. Where is the vision for fall prevention? J Am Geriatr Soc 2001; 49:676–677. 16. Brannan S, Dewar C, et al. A prospective study of the rate of falls before and after cataract surgery. Br J Ophthalmol 2003; 87(5):560–562. 17. Abdelhafiz AH, Austin CA. Visual factors should be assessed in older people presenting with falls or hip fracture. Age Ageing 2003; 32(1):26–30. 18. Jack CI, Smith T, et al. Prevalence of low vision in elderly patients admitted to an acute geriatric unit in Liverpool: elderly people who fall are more likely to have low vision. Gerontology 1995; 41(5):280–285. 19. Lord SR, Dayhew J, et al. Multifocal glasses impair edge-contrast sensitivity and depth perception and increase the risk of falls in older people. J Am Geriatr Soc 2002; 50(11):1760–1766. 20. Oghalai JS, Manolidis S, et al. Unrecognized benign paroxysmal positional vertigo in elderly patients. Otolaryngol Head Neck Surg 2000; 122(5):630–634. 21. Halmagyi GM, Curthoys IS, et al. The human horizontal vestibulo-ocular reflex in response to high-acceleration stimulation before and after unilateral vestibular neurectomy. Exp Brain Res 1990; 81(3):479–490. 22. Cremer PD, Halmagyi GM, et al. Semicircular canal plane head impulses detect absent function of individual semicircular canals. Brain 1998; 121(Pt 4):699–716.

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23. Della Santina CC, Cremer PD, et al. Comparison of head thrust test with head autorotation test reveals that the vestibulo-ocular reflex is enhanced during voluntary head movements. Arch Otolaryngol Head Neck Surg 2002; 128(9):1044–1054. 24. Oliva M, Martin Garcia MA, et al. The head-thrust test (HTT): physiopathological considerations and its clinical use in daily practice. Acta Otorrinolaringol Esp 1998; 49(4):275–279. 25. Powell LE, Myers AM. The Activities-Specific Balance Confidence (ABC) Scale. J Gerontol Ser A Biol Sci Med Sci 1995; 50(1):M28–34. 26. Myers AM, Powell LE, et al. Psychological indicators of balance confidence: relationship to actual and perceived abilities. J Gerontol Ser A Biol Sci Med Sci 1996; 51(1):M37–43. 27. Myers RL, Laenger CJ. Virtual reality in rehabilitation. Disability Rehabil 1998; 20(3):111–112. 28. Myers AM, Fletcher P, et al. Discriminative and evaluative properties of the ActivitiesSpecific Balance Confidence (ABC) Scale. J Gerontol 1998; 53A(4):M287–294. 29. Whitney SL, Hudak MT, et al. The activities-specific balance confidence scale and the dizziness handicap inventory: a comparison. J Vestib Res 1999; 9(4):253–259. 30. Jacobson GP, Newman CW. The development of the dizziness handicap inventory. Arch Otolaryngol Head Neck Surg 1990; 116(April):424–427. 31. Weber PC, Cass SP. Clinical assessment of postural stability. Am J Otol 1993; 14(6): 566–569. 32. Lord SR, Murray SM, et al. Sit-to-stand performance depends on sensation, speed, balance, and psychological status in addition to strength in older people. Gerontol A Biol Sci Med Sci 2002; 57(8):M539–543. 33. Maki BE, Perry SD, et al. Effect of facilitation of sensation from planter foot-surface boundaries on postural stabilization in young and older adults. J Gerontol Ser A Biol Sci Med Sci 1999; 54(6):M281–287. 34. Priplata AA, Niemi JB, et al. Vibrating insoles and balance control in elderly people. Lancet 2003; 362(9390):1123–1124. 35. Herdman SJ. Physical therapy management of vestibular disorders in older patients. Phys Ther Pract 1992; 1(1):77–87. 36. Nandapalan V, Smith CA, et al. Objective measurement of the benefit of walking sticks in peripheral vestibular balance disorders, using the sway weigh balance platform. J Laryngol Otol 1995; 109(9):836–840. 37. Jeka JJ. Light touch contact as a balance aid. Phys Ther 1997; 77(5):476–487. 38. Cohen HS, Kimball KT. Increased independence and decreased vertigo after vestibular rehabilitation. Otolaryngol Head Neck Surg 2003; 128(1):60–70. 39. Whitney SL, Wrisley DM, et al. The effect of age on vestibular rehabilitation outcomes. Laryngoscope 2002; 112(10):1785–1790. 40. Rose DJ, Clark S. Can the control of bodily orientation be significantly improved in a group of older adults with a history of falling? J Am Geriatric Soc 2000; 48: 275–282. 41. Shumway-Cook A, Gruber W, et al. The effect of multidimensional exercises on balance, mobility, and fall risk in community-dwelling older adults. Phys Ther 1997; 7(1):46–57. 42. Horak FB, Jonesrycewicz C, et al. Effects of vestibular rehabilitation on dizziness and imbalance. Otolaryngol Head Neck Surg 1992; 106(2):175–180. 43. Shepard NT, Telian SA. Programmatic vestibular rehabilitation. Head Neck Surg 1995; 112:173–182. 44. Strupp M, Arbusow V, et al. Vestibular exercises improve central vestibulospinal compensation after vestibular neuritis. Neurology 1998; 51(3):838–844.

ANNOTATED BIBLIOGRAPHY Lord SR, Dayhew J. Visual risk factors for falls in older people. J Am Geriatr Soc 2001; 49(5):508–515.

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This paper provides an excellent overview of the visual deficits that are commonly associated with falling in older persons. Oghalai JS, Manolidis S, et al. Unrecognized benign paroxysmal positional vertigo in elderly patients. Otolaryngol Head Neck Surg 2000; 122(5):630–634. The article describes a group of older adults who present to the clinic without complaints of dizziness or balance disorders. Nine percent had undiagnosed BPPV and 61% had dizziness when questioned.

10 Mal de Debarquement Timothy C. Hain and Dario A. Yacovino Departments of Physical Therapy, Neurology, and Otolaryngology, Northwestern University Medical School, Chicago, Illinois, U.S.A.

GENERAL DESCRIPTION OF THE CLINICAL PROBLEM Mal de debarquement (MDD), literally ‘‘sickness of disembarkment,’’ refers to inappropriate sensations of movement after exposure to motion. The syndrome typically follows a sea voyage, but similar sensations have been described following extended train travel, space flight, and after experience within a slowly rotating room (1,2). Symptoms usually include sensations of rocking and swaying accompanied by imbalance, but not rotational vertigo. MDD is distinguished from motion sickness, air sickness, simulator sickness, or seasickness (mal de mer) because subjects are predominantly symptom-free during the period of motion. MDD is distinguished from ‘‘landsickness’’ or post-motion vertigo by duration. Landsickness typically lasts less than 48 hours (3,4). We, like most other authors reporting on MDD, define it almost exclusively as a syndrome that persists for at least one month (4–7). It should be noted, however, that some authors refer to the very common short-lived, post-motion vertigo as MDD, and the longer duration form as ‘‘persistent MDD’’ (3). As of 2003, only 46 subjects with mal de debarquement as defined above have been described in the literature (4–7). However, this syndrome is probably more common than the literature might lead us to believe, as the level of awareness in the general population as well as in health personnel is very low. MDD occurs most commonly in middle-aged women (Fig. 1) but about 25% of reported cases of MDD have been in individuals over the age of 65. It has never been reported in children, and it is rarely encountered in persons in their 20s or 30s. The prevalence of vulnerability to MDD in the population is unknown.

BASIC SCIENCE Literature describing persons with persistent MDD is sparse. Brown and Baloh (5) reported six cases of MDD in 1987. They noted that despite anecdotal reference to this syndrome, there had been no published definition or description. Five of their six subjects were females, ranging in age from 38 to 71 years. Five of the subjects 125

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Figure 1 Age at onset of MDD syndrome, in decades. Source: From Ref. 7.

developed MDD after boat travel, with durations of exposure that varied from under five hours to 70 days. Subjects typically got little relief from antivertiginous medication. Three subjects exhibited a direction-changing positional nystagmus during electronystagmography. Caloric testing was normal in all. One patient had abnormally prolonged optokinetic afternystagmus, but the authors did not indicate whether this response was tested in all patients. Brown and Baloh suggested that MDD syndrome is caused by an abnormally persistent central nervous system (CNS) adaptation to the seagoing environment that causes a failure or delay of readaptation to the earth-stable environment. Murphy (6) reported four cases of MDD. All of these were female, ranging between 36 and 48 years of age. The duration of motion exposure ranged from eight hours to seven days, and the duration of symptoms between four weeks and one year. Three subjects developed MDD after exposure to water travel, the remaining patient after sleeping on a water bed. One patient had direction-changing positional nystagmus. Amitriptyline was helpful in one patient and lorazepam in another. Murphy suggests that the sensations described by subjects are consistent with a perception of abnormal linear acceleration, and that because the utricle is known to be responsible for responding to linear acceleration, MDD syndrome may result from ‘‘utricular dysfunction.’’ Murphy notes hormonal interaction with the vestibular system or brain, and/or inability of the brain to coordinate vestibular and ocular input as other possible causes. Mair (4) reported 10 cases of MDD. Again, all subjects were female, with ages ranging from 15 to 66 years. Sea travel provoked symptoms in eight and air travel in

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the remainder. Onset followed exposure variously from two hours to two days, with all but one in less than 18 hours. While one patient had a symptom duration of only three days, all others had symptoms persisting for at least one month, ranging up to two years. Mair suggested that defective or delayed readaptation following cessation of motion might be an important causative factor. Mair also noted that gender differences have been previously shown to exist in susceptibility to motion sickness and in the degree to which visual cues are used for orientation. Hain and associates (7) reported 27 cases of MDD. Nearly all were middleaged females (26/27, mean age 49.3). Duration of symptoms ranged from six months to 10 years (mean 3.5, SD 2.5). Symptoms were constant in 85%. Neither meclizine nor transdermal scopolamine was helpful. Benzodiazepines were of the most benefit. Balance rehabilitation physical therapy was undertaken by 15 patients, who on average reported a small benefit. Other authors have reported that a large percentage of persons disembarking from seagoing voyages experience a brief MDD syndrome, which by our definition would instead be termed ‘‘landsickness.’’ Gordon et al. (8) surveyed 234 healthy crew members of seagoing vessels. They reported a high incidence of a short-lived MDD symptom complex (73%). Presumably, the majority of their subjects were male. As a follow-up to that study, Gordon et al. (3) studied 116 male crew members of seagoing vessels. The subjects ranged in age from 18 to 33 years and sailed once or twice a week. Seventy-two percent reported having experienced MDD. The duration of symptoms ranged from one minute to two days, lasting less than six hours in 88% of the men. They found a significant relationship between susceptibility to seasickness and the occurrence of MDD. A similar short-lived syndrome has been reported after flight-simulator training of pilots (9). None of the subjects in the Gordon et al. study would qualify as having MDD according to our criteria. Similarly, Cohen (10) studied professional and amateur crew members participating in sailing on a 117-year-old sailing vessel. Cohen’s sample included 36 men and 23 women. After the first sailing day, 37% reported landsickness symptoms, increasing to 41% after the second day, and then decreasing to 20% after further experience. Males and females did not differ significantly in the incidence, intensity, or duration of symptoms. A number of other symptoms are reported by persons with MDD. Many suffer from migraine, ear pain, tinnitus, and ear fullness. A sensitivity to flashing lights and fluorescent lighting is commonly seen. Busy patterns can induce nausea. Also, symptoms of rocking may intensify while shopping in malls or grocery stores, or when walking in crowds. This probably reflects ‘‘visual dependence,’’ which is often associated with vestibular disorders (11). Ataxia is commonly seen during periods of stress and as fatigue increases throughout the day. Family situations can change as well as employment as these are impacted by the illness. Anxiety and depression are common. Most individuals recover, but many have reoccurrences. Some have a recurrent variant with reactivation following a plane ride, another boat trip, or a long car ride.

POTENTIAL ETIOLOGIC FACTORS IN MDD Given the consistency of the four studies on MDD, it would appear that, although landsickness may be common in both sexes following motion exposure, it is more

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likely to persist and become MDD in females. This has some implications regarding possible causes. Migraine The prevalence of migraine is estimated at 11% of the general population and about 20% in women during their reproductive years (12,13). Migraine has a similar female predominance at the age of 50 (3:1) when compared to MDD and occurs most commonly at the age of 35. Migraine has been strongly associated with motion sensitivity (14). From our data, almost one-fourth of the subjects fit the International Headache Society (IHS) criteria for migraine, with another almost 30% having recurring headaches (7). However, only one patient had a migraine on the MDDprovoking voyage, and in most of the subjects, the headaches have resolved. Studying a large group of migraine sufferers for MDD syndrome could help clarify the picture. Hormones There is a strong predominance of females in studies of MDD and also a relatively narrow age group. The predominance of females greatly exceeds that found in motion sickness among passengers at sea, where the ratio of females to males is about 5:3 (15). Anecdotal evidence suggests that MDD also lasts longer in women than in men. In our study, only about one-third were of postmenopausal ages and none were of premenarchal, suggesting that female hormones may facilitate this syndrome. Murphy (6) noted the age and female predominance in his subjects (4/4) and also postulated a hormonal effect. Based on this same evidence, one could also hypothesize that MDD is somehow related to factors biologically dependent on the presence of two copies of the X chromosome. On the other hand, landsickness clearly occurs in a large percentage of men after ocean voyages. These issues could be clarified by obtaining more data in this age group, studying males who are taking estrogen (e.g., for control of prostate cancer), and by further studies of MDD in large groups. Inner Ear Pathology, in Particular, Meniere’s Disease The symptomatology of MDD subjects can overlap with Meniere’s disease as many subjects complain of aural fullness, tinnitus, and hearing impairment (7). While no formal study has been done, it is possible that some patients with MDD actually have a variant of Meniere’s disease, which coincidentally flared up following motion exposure. Persistent CNS Adaptation to the Seagoing Environment MDD syndrome does not have the features of a ‘‘pathologic’’ disease, in the sense that it does not appear to follow an injury. Rather, it is provoked by exposure to motion that does not trouble most individuals, at least not in a persistent manner. Movements such as that experienced in a boat expose a person to rhythmic upward and downward movements along with tilts to the left and right. During this time, the brain must adjust leg and body motion so that they counter the

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rhythmic pattern of shipboard motion. Adaptation to such movement is sometimes called ‘‘gaining sea legs.’’ While the appearance of a rocking sensation as a result of adaptation might seem implausible, as it implies the initiation of an oscillating pattern, experimentally a pendular nystagmus lasting up to 60 minutes can be induced in rabbits by 24 hours of exposure to torsional or parallel swing stimuli (16). Psychogenic Etiology MDD does not appear to have a psychogenic basis. Several studies have noted that the relatively late age of onset as well as the lack of other medical complaints is not supportive of a somatization disorder (5,7). The extreme female predominance also is not consistent with a psychogenic etiology, which is generally more evenly distributed according to gender. History A typical case history is as follows: A 50-year-old woman went on her first ocean cruise. She had some motion sickness on the cruise, which responded to transdermal scopolamine. After returning from the cruise she developed ‘‘landsickness,’’ consisting of imbalance and a rocking sensation accompanied by fatigue and difficulty in concentrating. Her description was ‘‘imagine feeling like you are on rough seas 24 hours a day seven days a week.’’ The typical MDD patient presents with persistent landsickness after a sevenday ocean cruise. They complain of a constant rocking sensation that persists while awake. Frank vertigo (spinning) is unusual. Many patients with MDD report that they are able to drive, but that their symptoms return on getting out of the car. Normal persons may have landsickness for 48 hours, but MDD becomes likely with persistence beyond this time. Physical Examination The neurological and otological examination is usually normal, and the purpose of the physical examination is largely to exclude other entities. The tympanic membranes should be inspected for abnormality and hearing should be tested. The eye movements should be checked for nystagmus, impaired pursuit, and saccadic dysmetria or instability. Neurologically, balance should be assessed with the eyes-closed tandem Romberg test. Most patients with MDD are able to stand for six seconds, in spite of complaints of rocking and unsteadiness. Reflexes and motor power are expected to be normal. Coordination of the limbs, speech, and head should be normal. There should be no tremor, rigidity, or upper motor neuron signs. Diagnostic maneuvers for dizziness performed with the Frenzel goggles are often very helpful in differential diagnosis of vertigo (17). They should be performed in persons with MDD to exclude alternative diagnoses, particularly benign paroxysmal positional vertigo. There should be no spontaneous nystagmus. Positional testing is generally normal, but it may also sometimes reveal a low-amplitude direction changing positional nystagmus (5). Fistula testing should be performed in the clinic with Frenzel goggles if there is a history of SCUBA diving or onset of symptoms following an airplane flight.

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Figure 2 Duration of MDD symptoms. Source: From Ref. 7.

DIAGNOSIS OF MDD The diagnosis is made by a combination of the history (rocking after prolonged exposure to a boat) and exclusion of reasonable alternatives. A typical patient is a woman of appropriate age who has gone on a cruise and who has been rocking for at least a month (Figs. 1, 2). Symptoms that would make MDD less likely would be rotational vertigo, double vision, or bouncing vision. Table 1 outlines our recommendations for diagnostic testing. Because of the high prevalence of aural fullness, tinnitus, and subjective hearing loss in MDD, tests to exclude Meniere’s disease should be done, and if there is a history of plane flight, perilymph fistula should also be considered (7). The electronystagmography (ENG) test result is usually normal, but occasionally it may find unusually strong or prolonged optokinetic afternystagmus or vestibular responses, and there is also

Table 1 Recommended Diagnostic Tests to Exclude Alternative Causes of Ataxia Audiogram ENG ECOG if hearing is abnormal MRI of brain if neurological examination is abnormal Blood tests to exclude alternative causes (CBC, blood glucose, FTA, B12) Abbreviations: ENG, electronystagmography; ECOG, electrocohleography; MRI, magnetic resonance imaging; FTA, fluorescent treponema antibody.

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Table 2 Medications Probably Ineffective in MDD Dramamine Meclizine Scopolamine

sometimes direction-changing positional nystagmus, as has been reported by Brown and Baloh (5). Blood testing is normal in MDD. Nevertheless, blood testing for treatable causes of unsteadiness, such as B12 deficiency, syphilis, hypoglycemia, and anemia are recommended, to exclude other disorders. Magnetic resonance imaging (MRI) testing is nearly always normal in persons with MDD, but it is indicated in persons with central findings on neurological examination such as nystagmus or limb ataxia.

TREATMENT OF MDD We will consider both treatments aimed at preventing MDD and treatments aimed at ameliorating the effects of MDD once it has occurred. If MDD is indeed related to inappropriate vestibular adaptation, medications that slow such adaptation (vestibular suppressants, benzodiazepines) might be tried. We know of no studies to date regarding the prevention of MDD through medication; patients with the recurrent variant of this condition have indicated that low doses of klonapin or lorazepam taken prior to the anticipated motion exposure are often effective. Anticholinergic medications such as scopolamine do not appear to be effective for prevention (7). Antihistamines such as meclizine are also ineffective (Table 2) (7). Certainly, for persons with a history of MDD, avoidance of further prolonged exposure to rocking motion seems prudent. With respect to treatments of an ongoing MDD syndrome, there is evidence of a positive effect of benzodiazepines and amitriptyline (Table 3) (7). Meclizine and scopolamine were entirely ineffective. Occasionally patients have reported a good response to diverse medications such as gabapentin and carbamazepine. Finally, Brown and Baloh (5), Murphy (6), and Mair (4) all recommend vestibular rehabilitation or exercise for the MDD syndrome, although no results for this intervention were reported. Zimbelman and Walton reported a case of MDD that may have benefited from vestibular rehabilitation (18). However, no formal study has been done proving its efficacy. In addition to vestibular rehabilitation therapy, medications that speed adaptation, for example, stimulants such as the amphetamine family, might be tried.

Table 3 Treatment of Ongoing Symptoms of MDD with Benzodiazepines Drug

Dose (mg)

Interval

Adverse effects Sedation Pregnancy cat D Sedation Pregnancy cat D

Diazepam

2

BID

Klonapin

0.5

BID

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Although there is anecdotal evidence that stimulants may be helpful, again, these medications have not been formally studied for MDD.

ALGORITHM Figure 3 illustrates a decision tree regarding MDD. The diagnosis should be considered in a person who presents with a persistent rocking sensation following a prolonged movement exposure, such as a seven-day cruise. MDD is probable if the duration of symptoms is at least one month and alternative causes of dizziness have been excluded.

Figure 3 Decision tree for MDD showing progression from presenting symptoms through physical examination and testing to treatment. Abbreviations: MRI, magnetic resonance imaging; ENG, electronystagmography; FTA, fluorescent treponema antibody.

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SUMMARY In summary, persistent MDD is a prolonged sensation of rocking, swaying, imbalance, or other motion typically triggered by a seagoing voyage. The disorder appears to occur almost exclusively in females, with age of onset typically in the 40s. The cause is unclear. Symptoms usually diminish with time, but can last for years. Certainly, more work is required in order to obtain a greater understanding of this little-known disorder. An increased awareness among physicians may help lead to the clinical knowledge necessary to develop effective treatment strategies. ACKNOWLEDGMENTS We gratefully acknowledge the assistance of the MDD support group in writing this review article. REFERENCES 1. Stott J. Adaptation to nauseogenic motion stimuli and its application in the treatment of airsickness. In: GH C, ed. Motion and Space Sickness. Boca Raton: CRC Press, 1990. 2. Graybiel A. Structural elements in the concept of motion sickness. Aerospace Med 1969; 40:351–367. 3. Gordon CR, Spitzer O, Doweck I, Melamed Y, Shupak A. Clinical features of mal de debarquement: adaptation and habituation to sea conditions. J Vestib Res 1995; 5(5):363–369. 4. Mair I. The mal de debarquement syndrome. J Audiol Med 1996; 5:21–25. 5. Brown JJ, Baloh RW. Persistent mal de debarquement syndrome: a motion-induced subjective disorder of balance. Am J Otolaryngol 1987; 8(4):219–222. 6. Murphy TP. Mal de debarquement syndrome: a forgotten entity? Otolaryngol Head Neck Surg 1993; 109(1):10–13. 7. Hain TC, Hanna PA, Rheinberger MA. Mal de debarquement. Arch Otolaryngol Head Neck Surg 1999; 125(6):615–620. 8. Gordon CR, Shupak A, Nachum Z. Mal de debarquement. Arch Otolaryngol Head Neck Surg 2000; 126(6):805–806. 9. Ungs TJ. Simulator induced syndrome: evidence for long-term after effects. Aviat Space Environ Med 1989; 60:252–255. 10. Cohen H. Mild mal de debarquement after sailing. Ann N Y Acad Sci 1996; 781:598–600. 11. Guerraz M, Yardley L, Bertholon P, et al. Visual vertigo: symptom assessment, spatial orientation and postural control. Brain 2001; 124:1646–1656. 12. Stewart WF, Lipton RB. Migraine headache: epidemiology and health care utilization. Cephalalgia 1993; 13(suppl 12):41–46. 13. Stewart WF, Shechter A, Rasmussen BK. Migraine prevalence. A review of populationbased studies. Neurology 1994; 44(6 suppl 4):S17–S23. 14. Kayan A, Hood JD. Neuro-otological manifestations of migraine. Brain 1984; 107(Pt 4): 1123–1142. 15. Lawther A, Griffin MJ. A survey of the occurrence of motion sickness amongst passengers at sea. Aviat Space Environ Med 1988; 59:399–406. 16. Kleinschmidt HJ, Collewijn H. A search for habituation of vestibulo-ocular reactions to rotatory and linear sinusoidal accelerations in the rabbit. Exp Neurol 1975; 47(2):257–267. 17. Hain T. Approach to the Vertigo Patient. In: Biller J, ed. Practical Neurology. Philadelphia: Lippincott-Raven, 1997. 18. Zimbelman JL, Watson TM. Vestibular rehabilitation of a patient with persistent mal de debarquement. Physical Therapy Case Reports 1992; 2:129–133.

11 Meniere’s Disease in the Elderly Ronen Perez and Julian M. Nedzelski Department of Otolaryngology and Head and Neck Surgery, Sunnybrook and Women’s College Health Sciences Centre and University of Toronto, Toronto, Ontario, Canada

Meniere’s disease is a disorder of the inner ear, which is characterized by vertiginous episodes, fluctuating hearing loss, and tinnitus. Although the disorder classically develops in middle age (peak 35–50 years), it is not uncommon in patients over 65 years old.

EPIDEMIOLOGY The incidence and prevalence of Meniere’s disease is reported to widely vary. While the incidence reported in the United Kingdom is 157 per 100,000, in France it is only eight per 100,000 (1). Since Meniere’s disease is extremely rare in the pediatric population, the true incidence in the adult population is probably slightly higher (2). In a detailed thorough clinical study, Ballester et al. (3) reported that 15.3% of 432 active Meniere’s patients were over 65. Sixty percent of these individuals had the initial presentation over the age of 65 and the remaining 40% presented with reactivation of long-standing disease. The authors of this chapter report approximately similar numbers with 9% of patients presenting with the disease over the age of 65 (4). There are conflicting reports regarding sexual preponderance of the disease with respect to the full range of the population (5–8). While Ballester’s study reported a clear female preponderance in the elderly population (46 women and 20 men), our data do not support this. Published reports regarding the frequency of bilateral Meniere’s disease vary from 2% to 78% of patients (9,10). The results of an ongoing study at the authors’ institution seem to indicate that while the percentage of clinical Meniere’s disease of the contralateral ear is relatively low, the percentage of patients with a contralateral low frequency hearing loss is higher. Kitahara et al. (11) reported that the percentage of bilateral involvement at the onset of the disease was higher in patients over 60.

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Familial occurrence of the disease is also unclear but has been described in 14% of patients in a Swedish study and 5% of patients in a study conducted in the United Kingdom (12,13).

PATHOGENESIS The widely accepted histopathologic changes associated with Meniere’s disease relate to the membranous labyrinth. The hallmark of this disturbance is endolymphatic hydrops (14,15). Over-accumulation of endolymph is believed to cause anatomical distortion of the membranous labyrinth (16). Although it is widely known that hypertension is common among the elderly population, to the best of our knowledge there is no evidence in the literature to suggest a link between hypertension and the endolymphatic hydrops felt to be a feature of Meniere’s. Endolymph is produced by the stria vascularis in the cochlea and by dark cells scattered throughout the vestibular labyrinth. It is absorbed within the endolymphatic sac as well as the stria vascularis. Numerous mechanisms for the development of hydrops have been proposed. These include obstruction of the endolymphatic duct, dysfunction of the endolymphatic sac, overproduction of endolymph, infection, immune-mediated etiologies, and vascular disturbance (16–21). A breakdown in the barrier between endolymph and perilymph is thought to be the significant event in the pathophysiology of the disease, especially in the acute attacks. The range of this barrier disturbance is thought to vary between a rupture to a small leakage (22–24). The rupture or leakage leads to potassium intoxication of the hair cells. This reversible hair cell depolarization transiently interferes with the mechanoelectrical transduction of these structures. This explains the sudden tinnitus increase, hearing loss, and vertigo (25). It has been shown that the membranous labyrinth easily heals restoring the normal potassium balance and consequently terminating the acute exacerbation (26). Repeated exposure of the hair cells to the high concentration of potassium is thought to cause the gradual deterioration in hearing and vestibular function (27). It has been shown histologically that the hair cells and the nerves remain unchanged in these patients. A decrease in the hair cell or neuronal population occurs rarely and only in the severe cases of the disease (11). In the past decade, several studies have implicated an immune response as being responsible for endolymphatic hydrops (20,28–30). Known immune-mediated diseases such as Cogan’s, and polyartheritis nodosa are known to cause inner ear disturbances (31,32). Circulating immune complexes were found in the serum of patients with Meniere’s (30). C3 and C1q complement have been shown to be elevated in patients with the disease as have circulating serum antibodies to heat shock protein 70 (29,33). In addition, animal models of endolymphatic hydrops induced by a secondary immune response have been described (34). The vascular etiology is of special interest with regard to the elderly population. The possibility of a common vascular mechanism for migraine headache and Meniere’s syndrome has been proposed (6,35). Some studies suggest that altered microcirculation and venous drainage may be significant in the pathophysiology of Meniere’s disease (21,36). Kimura et al. (37) postulated that endolymphatic hydrops may be a result of interference with the blood supply to the endolymphatic sac causing alteration in its function. The peak incidence of the disease in middle-aged patients does not support this theory.

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DIAGNOSIS History A well-taken history is paramount for the diagnosis of Meniere’s disease. Typically, Meniere’s patients present with episodes of severe vertigo, tinnitus, and ipsilateral hearing loss (8). The duration of the episode is from minutes to hours, most commonly two to three hours. The vertiginous episodes may appear suddenly without warning although generally are preceded by increasing tinnitus and aural fullness (38). Approximately 60% of elderly patients diagnosed as having Meniere’s have their initial episode at age 65 or older. The remaining 40% have an acute reactivation of long-standing disease (3). Drop attacks are a rare occurrence in patients afflicted with Meniere’s disease and consist of sudden falls without loss of consciousness or vertigo. They are thought to occur as a consequence of sudden alteration of proprioception by the extremity antigravity muscles. Patients often report a sensation of being ‘‘pushed to the ground by a giant hand.’’ Tumarkin (39) described these attacks in 1936 and attributed them to acute otolithic dysfunction (40). Current thinking is that an inappropriate postural adjustment occurs as a result of an acute change in otolithic output (41). Less than 5% of Meniere’s patients suffer from drop attacks during the course of their disease. Importantly, as many as 25% of patients, initially presenting with Meniere’s symptoms over age 65, suffered from drop attacks (3). This is a significantly higher number than in the general Meniere’s population. This observation is of importance for physicians treating the geriatric population for two main reasons: namely, the differential diagnosis with a possible vascular event often leads to an erroneous diagnosis. Secondly, the drop attacks may be associated with fractures, which have a tremendous impact on all aspects of life of the patients. The sensorineural hearing loss associated with Meniere’s typically fluctuates at onset of the disease. Additionally, in contrast to most other causes of sensorineural hearing loss (noise, age, ototoxicity), the hearing loss associated with Meniere’s typically begins in the low frequencies (Fig. 1) (38). Meniere’s disease in most cases is progressive, leading to a permanent hearing loss. Over time, the audiogram assumes a flat configuration. It is well known that a majority of the elderly have varying degrees of hearing loss. The typical audiometric configuration associated with presbycusis is a symmetric downsloping sensorineural hearing loss (Fig. 2). The acute and chronic hearing loss associated with Meniere’s in the elderly is superimposed on the presbycutic loss (Fig. 3). Because the low frequencies are most affected in early Meniere’s disease and these are typically spared in presbycusis, afflicted elderly patients are especially handicapped in the involved ear early. Finally, because hearing loss, tinnitus, and balance-related complaints are commonly experienced by the elderly, the diagnosis of Meniere’s is made difficult.

Physical Examination As is the case in the general population, the physical examination in elderly Meniere’s patients, apart from an asymmetric hearing loss, is normal. The diagnosis is primarily based on the history. A full and thorough ear, nose, and throat (ENT) and neuro-otologic exam should be performed. This by definition includes examination of all of the cranial nerves, and cerebellar as well as gait assessment. There are a

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Figure 1 A typical audiometric profile in early Meniere’s disease.

number of considerations in the geriatric population which need to be made. Special attention should be given to ruling out orthostatic hypotension. Orthostatic hypotension, a common finding in the elderly, may be described as a sensation of transient vertigo. The neck and skull should be carefully auscultated for bruits, which are barometers of vascular disease. The presence of nystagmus without vertigo is suspicious for central pathology and warrants further prompt investigation.

Figure 2 A typical audiometric profile for presbycusis.

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Figure 3 An audiometric illustration of superimposed Meniere’s disease and presbycusisrelated hearing loss.

Investigations As stated earlier, there is no diagnostic test for Meniere’s disease. All tests should be viewed as corroborative. Audiometric Evaluation The typical sensorineural hearing loss associated with early onset Meniere’s disease is primarily in the low frequencies and this finding contributes significantly to establishing the diagnosis. In the elderly, this is typically superimposed on the loss as a consequence of presbycusis. Both the hearing loss noted as a consequence of aging (presbycusis) and that of Meniere’s disease can lead to a very poor quality of hearing, that is to say, the ability to discriminate especially conversations. The fact that this can dramatically fluctuate in the Meniere’s afflicted ear makes it doubly frustrating for the elderly (42). Electrocochleography The summating potential in Meniere’s patients is larger and with a higher negativity. This is a reflection of the distension of the basilar membrane into the scala vestibuli (43). The difficulty in obtaining reproducible recordings and the variability of the wave amplitude in different age groups limits the diagnostic use of this test in Meniere’s patients. Otoacoustic Emissions Their value in the diagnosis of Meniere’s is still questionable. Of special interest is the finding of smaller-amplitude emissions in the opposite ear. This may bear implications regarding impending disease in the contralateral ear.

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Electronystagmography The caloric test, one part in the battery of the electronystagmography tests, is useful in corroborating the affected ear. In 48–73% of patients with Meniere’s disease a significant caloric response reduction is noted in the affected ear (44,45). An absent caloric response is reported in 6–11% of patients.

Differential Diagnosis Meniere’s disease in the elderly is relatively rare. However, given the higher incidence of drop spells in this patient population, it does pose as a consideration in the diagnosis of cerebrovascular disease. In Ballaster’s study, five of the 39 patients with Meniere’s initially diagnosed at age 65 or older (13%) were misdiagnosed as having a stroke because of the associated drop spells. Another important differential diagnosis in the elderly patients is vertebrobasilar insufficiency (VBI). Sudden vertigo is the most common symptom of VBI in older patients. Approximately 50–60% of patients with VBI have spinning vertigo as their initial complaint (46). The vertigo usually lasts minutes and is often associated with nausea and vomiting. In all cases, it is associated with additional symptoms of brainstem involvement, thereby excluding the diagnosis of Meniere’s. Although benign paroxysmal positional vertigo (BPPV) is easily differentiated from Meniere’s (especially by otolaryngologists), it is worth mentioning because it is the most common cause of peripheral vertigo and is common in the elderly. In BPPV, the vertigo typically lasts for seconds and always less than a minute. It occurs when assuming a certain head position typically with the involved ear undermost after rolling over in bed. In contrast to Meniere’s disease, it is not associated with hearing loss or tinnitus. The nystagmus evoked in BPPV by the Dix-Hallpike maneuver has features, which are pathognomonic of the disease (47). That is to say, the nystagmus is rotatory, beating toward the undermost ear, delayed in onset, lasts for seconds, and is associated with marked symptoms of spinning and nausea. The term ‘‘presbyvertigo’’ is not uncommonly used to describe an ill-defined sense of poor balance by elderly patients. They do not experience vertigo as such, do not realize any associated fluctuating aural symptoms nor do they experience any transient neurologic deficits. It is assumed that these symptoms are a consequence of combined vestibular hair cell loss, declining vision, and compromised proprioception.

TREATMENT To date, there is no curative treatment for Meniere’s disease. All treatment modalities are aimed at relieving the main symptoms, notably the debilitating vertigo with its associated nausea. Definitive treatment for vertigo and Meniere’s associated drop attacks consist of vestibular ablation for the involved ear. It is our experience and Ballester’s that the elderly show more tolerance to the vertigo attacks than the general population. Once the patients are reassured that the disease is not life threatening, they tolerate the vertigo quite well. This is not the case with regard to the drop attacks, which are extremely debilitating and potentially fatal. The hearing loss and tinnitus are also debilitating and as mentioned previously are superimposed on presbycusis causing significant hearing handicap.

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Scheme 1 The diagnosis and management flowchart.

The first line of medical treatment consists of salt restriction and diuretic therapy (48). At the dizziness clinic at Sunnybrook, hydrochlorothiazide is usually initially prescribed. A relatively large percentage of elderly patients are already under medical treatment for hypertension, which in some cases includes a diuretic.

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Collaboration with the patient’s family physician is sometimes required for possible modification of antihypertensive treatment to include a diuretic. It is important to monitor potassium levels. The rationale for this is to reduce endolymph volume. There are conflicting reports in the literature regarding the efficacy of this treatment (49,50). Betahistine (serc) and other vasodilators have been used. They are given in the belief that Meniere’s disease is a result of strial ischemia. The intent of their administration is to improve the blood flow to the labyrinth. Several studies show reduction of vertiginous attacks with these drugs, but again clear-cut efficacy has not been shown (51,52). These medications are very well tolerated by the elderly population with few side effects. In many cases, symptomatic relief is sought by stronger anti-vertiginous medications, antiemetics, and sedatives. These drugs are not always tolerated well and in some cases cause depression in the elderly. Their use should be carefully monitored. The emergence of chemical ablation in the last two decades has reduced the percentage of patients undergoing surgical procedures (53,54). Numerous protocols of gentamicin administration have been described. The protocol used at Sunnybrook involves insertion of a T-tube attached to a catheter in the clinic under local anesthesia. Three doses of gentamicin daily over four days are administered. Approximately 10% of the patients who underwent this treatment during the last 15 years were over 65 at the time of the treatment. Complete control of vertigo was achieved in 85% of individuals. Hearing was worse in 26%, unchanged in 48%, and improved in 26% (4). Control of vertigo in the elderly group was similar to younger individuals. However, the percentage of elderly individuals who realized a worsening of hearing was higher (37% vs. 26%). In selected cases, notably patients experiencing drop attacks, labyrinthectomy may be the preferred treatment option. Other forms of surgical treatment include endolymphatic sac compression, cochleosacculotomy, and vestibular neurinectomy (55–57). Dayal and Proctor (58) specifically addressed labyrinthectomy for Meniere’s in the elderly and concluded that while taking into account the chronologic as well as the physiologic age of the patient, labyrinthectomy is recommended for patients with incapacitating vertigo without serviceable hearing. In all cases of deliberate ablation of peripheral vestibular function, it is imperative that patients undergo a course of intensive vestibular rehabilitation exercises.

SUMMARY Although the incidence of Meniere’s disease is thought to peak at middle age, its occurrence in patients over age 65 is not uncommon. The disease in the elderly may present initially over the age of 65 or may be a consequence of long-standing disease. Drop attacks in the elderly are a more frequent occurrence. They can be extremely debilitating and may result in trauma and fractures. Meniere’s disease in the elderly is often confused with vertebrobasilar insufficiency. The hearing loss associated with the disease is frequently superimposed on presbycusis and leads to a more significant handicap. Caution should be practiced when using medications, which may be poorly tolerated by the elderly, i.e., sedatives. On the other hand, these patients frequently are a high surgical risk. Finally, although vertigo control is equal to that of younger individuals, hearing loss as a consequence of middle ear gentamicin instillation is higher in the elderly.

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REFERENCES 1. Pfaltz CR, Thomsen J. Symptomatology and definition of Meniere’s disease. In: Pfaltz CR, ed. Controversial Aspects of Meniere’s Disease. New York: Georg Thieme, 1986. 2. Wladislavoski-Waserman P, Facer GW, Mokri B, Kurland LT. Meniere’s disease: a 30-year epidemiologic and clinical study in Rochester, MN, 1951–1980. Laryngoscope 1984; 94:1098–1102. 3. Ballester M, Liard P, Vibert D, Hausler R. Meniere’s disease in the elderly. Otol Neurotol 2002; 23:73–78. 4. Kaplan DM, Nedzelski JM, Chen JM, Shipp DB. Intratympanic gentamicin for the treatment of unilateral Meniere’s disease. Laryngoscope 2000; 110:1298–1305. 5. Watanabe I. Meniere’s disease in males and females. Acta Otolaryngol (Stockh) 1981; 91:511–514. 6. Parker W. Meniere’s disease. Etiologic considerations. Arch Otolaryngol Head Neck Surg 1995; 121:377–382. 7. Oosterveld WJ. Meniere’s disease, signs and symptoms. J Laryngol Otol 1980; 94: 885–894. 8. Paparella MM. The cause (multifactorial inheritance) and pathogenesis (endolymphatic malabsorbtion) of Meniere’s disease and its symptoms (mechanical and chemical). Acta Otolaryngol (Stockh) 1985; 99:445–451. 9. Balkani T, Sizes b, Arenberg I. Bilateral aspects of Meniere’s disease: an underestimated clinical entity. Otolaryngol Clin North Am 1980; 13:603–609. 10. Stahle J, Friedberg U, Svedberg A. Long term progression of Meniere’s disease. Acta Otolaryngol (Stockh) Suppl 1991; 485:78–83. 11. Kitahara M, Matsubara H, Takeda T, Yazawa Y. Bilateral Meniere’s disease. Adv Otorhinolaryngol 1979; 25:117–121. 12. Birgerson L, Gustavson KH, Stahle J. Familial Meniere’s disease: a genetic investigation. Am J Otol 1987; 8:323–326. 13. Morrison AW. Meniere’s disease. J R Soc Med 1981; 74:183–189. 14. Anatoli-Candela F. The histopathology of Meniere’s disease. Acta Otolaryngol (Stockh) Suppl 1976; 340:1–42. 15. Schuknecht HF, Igarishi M. Pathophysiology of Meniere’s disease. In: Pfaltz CR, ed. Controversial Aspects of Meniere’s Disease. New York: Georg Thieme, 1986. 16. Paparella MM, Mancini F. Vestibular Meniere’s disease. Otolaryngol Head Neck Surg 1985; 93:148–151. 17. Kimura RS, Schucknecht HF. Membranous hydrops in the inner ear of the guinea pig after obliteration of the endolymphatic sac. Pract Otorhinolaryngol 1965; 27:343–354. 18. Henriksson N, Igarishi M, Tonndorf J. Pathophysiology of Meniere’s disease. In: Pfaltz CR, ed. Controversial Aspects of Meniere’s Disease. New York: Georg Thieme, 1986. 19. Fukuda S, Keithley EM, Harris JP. The development of endolymphatic hydrops following CMV inoculation of the endolymphatic sac. Laryngoscope 1988; 98:439–443. 20. Derebery MJ, Rao VS, Siglock TJ, Linthicum FH, Nelson RA. Meniere’s disease: an immune complex-mediated illness? Laryngoscope 1991; 105:225–229. 21. Gussen R. Vascular mechanisms in Meniere’s disease. Otolaryngol Head Neck Surg 1983; 91:68–71. 22. Kimura RS. Fistulae in the membranous labyrinth. Ann Otol Rhinol Laryngol 1984; 93(suppl 112):36–43. 23. Schucknecht HF. Meniere’s disease. Otolaryngol Clin North Am 1968; 1:331. 24. Jahnke K. Permeability barriers of the inner ear in respect to the Meniere’s attack. In: Vosteen KH, ed. Meniere’s Disease: Pathogenesis, Diagnosis, and Treatment. Stuttgart: Georg Thieme, 1981. 25. Zenner HP, Ruppersberg JP, Lowenheim H, Gummer AW. Pathophysiology: transduction and motor disturbances of hair cells by endolymphatic hydrops and transitory

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29. 30. 31. 32. 33. 34. 35. 36. 37. 38. 39. 40. 41. 42. 43.

44. 45.

46. 47. 48. 49. 50.

Perez and Nedzelski endolymph leakage. In: Harris JP, ed. Meniere’s Disease. The Hague: Kugler Publications, 1999. Kimura RS, Schucknecht HF. Effect of fistulae on endolymphatic hydrops. Ann Otol 1975; 84:271–286. Thomsen J, Bretlau P. General conclusions. In: Pfaltz CR, ed. Controversial Aspects of Meniere’s Disease. Stuttgart: Georg Thieme, 1986. Zanneti FR, Plester D, Klein R, Bursa-Zanneti Z, Berg PA. Immunological patterns of inner ear disease including Meniere’s disease. In: Nadol JB Jr, ed. Meniere’s Disease. Ansterdam/Berkely/Milano: Kugler and Ghedini Publ, 1989:133–137. Hausler R, Arnold W, Schifferli J. C3 and C1q complement deposits in the membranous labyrinth of patients with Meniere´s disease. Adv Otorhinolaryngol 1988; 42:116–122. Brookes GB. Circulating immune complexes in Meniere’s disease. Arch Otol Laryngol Head Neck Surg 1986; 112:536–540. Schucknecht HF, Nadol JB Jr. Temporal bone pathology in a case of Cogan’s syndrome. Laryngoscope 1994; 104:1135–1142. Jenkins HA, Pollak AM, Fisch U. Polyarteritis nodosa as a cause of sudden deafness: a human temporal bone study. Am J Otolaryngol 1981; 2:99–107. Rauch SD, San Martin JE, Moscicki RA, Bloch KJ. Serum antibodies against heat shock protein 70 in Meniere’s disease. Am J Otol 1995; 16:648–652. Tomiyama S. Development of endolymphatic hydrops following immune response in the endolymphatic sac of the guinea pig. Acta Otolaryngol (Stockh) 1992; 112:470–478. Kayan A, Hood JD. Neuro-otological manifestation of migraine. Brain 1984; 107: 1123–1142. Gussen R. Vascular mechanisms in Meniere’s disease. Theoretical considerations. Arch Otolaryngol 1982; 108:544–549. Kimura RS, Trehey JA, Hutta J. Degeneration of vestibular sensori cells caused by ablation of the aqueduct in the gerbil ear. Ann Otol Rhinol Laryngol 1995; 104:155–160. Barber HO. Meniere’s disease: symptomatology. In: Oosterveld WJ, ed. Meniere’s Disease: A Comprehensive Appraisal. New York: John Wiley, 1983. Tumarkin A. The otolithic catastrophe: a new syndrome. Br Med J 1936; i:175. Pillsbury HC III, Postma DS. Lermoyez’ syndrome and the otolithic crisis of Tumarkin. Otolaryngol Clin North Am 1983; 16:197–203. Odkvist LM, Bergenius J. Drop attacks in Meniere’s disease. Acta Otolaryngol (Stockh) Suppl 1988; 455:82–85. Morrison AW. Diagnostic and laboratory evaluation in Meniere’s disease. In: Harris JP, ed. Meniere’s Disease. The Hague: Kugler Publications, 1999. Morrison AW, Moffat DA, O’Connor AF. Clinical usefulness of electrocochleography in Meniere’s disease: an analysis of dehydrating agents. Otolaryngol Clin North Am 1980; 13:703–721. Black O, Kitch R. A review of vestibular test results in Meniere’s disease. Otolaryngol Clin North Am 1980; 13:631–642. Stahle J, Klockhoff I. Diagnostic procedures, differential diagnosis and general conclusions. In: Pfaltz CR, ed. Controversial Aspects of Meniere’s Disease. New York: Georg Thieme, 1986. Baloh RW. Vertebrobasilar insufficiency and stroke. Otolryngol Head Neck Surg 1995; 112:114–117. Epley J. New dimensions of benign positional vertigo. Otolaryngol Head Neck Surg 1980; 88:599–605. Jackson CG, Glasscock ME III, Davis WE, Hughes GB, Sismanis A. Medical management of Meniere’s disease. Ann Otol 1981; 90:142–147. Klockhoff I, Lindblom U. Meniere’s disease and hydrochlorothiazide—a critical analysis of symptoms and therapeutic effects. Acta Otolaryngol (Stockh) 1967; 63:347–365. Klockhoff I, Lindblom U, Stahle J. Diuretic treatment of Meniere’s disease. Arch Otolaryngol 1974; 100:262–265.

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51. Wilmot TJ, Menon GN. Betahistine in Meniere’s disease. J Laryngol Otol 1976; 90: 833–840. 52. Segers JM, Bedts D. Clinical trials of betahistine hydrochloride in the treatment of Meniere’s disease. Acta Otorhinolaryngol Belg 1975; 29:814–821. 53. Nedzelski JM, Bryce GE, Pfeiderer AG. Treatment of Meniere’s disease with topical gentamicine: a preliminary report. J Otolaryngol 1992; 21:94–101. 54. Nedzelski JM, Schessel DA, Bryce GE. Chemical labyrinthectomy: local application of gentamicin for the treatment of unilateral Meniere’s disease. Am J Otol 1992; 13:18–22. 55. Glasscock ME III, Jackson CG, Poe DS, Johnson GD. What do I think of sac surgery in 1989. Am J Otol 1989; 10:230–233. 56. Schuknecht HF. Cochleosacculotomy for Meniere’s disease: theory, technique and results. Laryngoscope 1982; 92:853–858. 57. Silverstein H, Norrell H, Rosenberg S. The resurrection of vestibular neurinectomy: a 10 year experience with 115 cases. J Neurosurg 1990; 72:533–539. 58. Dayal VS, Proctor T. Labyrinthectomy in the elderly. Am J Otol 1995; 16:110–114.

ANNOTATED BIBLIOGRAPHY Ballester M, Liard P, Vibert D, Hausler R. Meniere’s disease in the elderly. Otol Neurotol 2002; 23:73–78. This is a particularly good reference. It is thorough and detailed and to our knowledge is the only paper specifically dealing with Meniere’s disease in the geriatric population.

12 Dizziness in the Elderly David E. Eibling Department Otolaryngology—Head and Neck Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION Dizziness is a common symptom that affects more than 30% of elderly individuals. In a study of 1622 elderly patients, dizziness accounted for one-third of all visits to primary care physicians for those older than 65, and was the most common complaint for those older than 75 (1). Seven percent of primary care visits by elderly patients were for symptoms of dizziness (2). Diagnosis of the underlying etiology and directed therapy is mandatory, since dizziness due to any number of causes can lead to falls with serious consequences in the geriatric population. Fear of falling results in disproportionate reductions in quality of life for the elderly, with nearly one-half of elderly dizzy patients reporting that they restrict their activities due to fear of falling versus 3% of age-matched controls (3). The symptom of dizziness is often assumed by the patient, as well as the caregiver, to be due to a primary disorder of the vestibular end organ. However, many causes of dizziness arise from disorders of other organ systems, especially the cardiovascular and non-vestibular neurologic systems. Sloane points out that most dizziness is non-vestibular. Failure to correctly identify the cause will result in persistent symptoms and ineffective therapy. The challenge for the evaluating physician is to first rule out life-threatening causes of dizziness such as a cardiac arrhythmia or brainstem stroke, to then narrow the list of diagnoses to the most probable etiology(s), and then optimize treatment. An incomplete listing of some of the causes of non-vestibular dizziness that may be encountered in elderly dizzy patients are listed in Table 1. Tinetti et al. (4) have proposed that dizziness in the elderly be considered a ‘‘geriatric syndrome’’ since dizziness is so prevalent in this population. In editorial remarks to Tinetti et al.’s paper, Drachman (5) expressed his concerns that the use of this term suggests that dizziness is an inevitable component of the aging process and reduces the incentive of the physician to seek a treatable cause. Indeed, Sloane et al. (1) point out that the cause(s) of dizziness in the elderly are often identifiable, and treatable.

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Table 1 Some Non-vestibular Causes of Dizziness in the Elderly Listed in Order of Decreasing Severity Life-threatening, possibly immediately Cardiac arrhythmia Cardiac failure Shock Hypoxia Impending seizure Incipient stroke Serious, but not likely to be immediately life-threatening Anemia Vertebrobasilar insufficiency Subclavian Steal Cerebellar infarction Orthostatic hypotension Loss of volume (hypovolemia) Blood, fluid loss, diuretics Loss of vascular tone Autonomic dysfunction Prolonged bed rest Antihypertensive therapy Corticosteroid deficiency Iatrogenic or due to disease Loss of cardiac responsiveness Cardiac disease Chronotopic incompetence Medication Pacemaker Central lesion Encephalitis Degenerative disease Brain tumor Toxin exposure Affecting mobility and quality of life Disequilibrium (may be vestibular) Disequilibrium of aging Multi-sensory deprivation Muscle weakness Sarcopenia CVA Spinal stenosis Central Parkinsons’s disease Orthostatic tremor Degenerative disease Drugs Alcohol Sedatives CNS active medications Brain lesion Encephalitis Brain tumor Chronic degenerative disease

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TAXONOMY OF DIZZINESS IN THE ELDERLY More than 30 years ago (prior to the availability of noninvasive intracranial and vascular imaging), Drachman and Hart (6) suggested that the complaint of dizziness could be categorized based on symptom description. Current understanding of balance function and its disorders, as well as readily available diagnostic studies, permit more accurate identification of the underlying etiology and selection of more appropriate therapeutic modalities. A careful history and focused physical examination will usually suggest one or more probable diagnoses, even in the elderly. Unfortunately, vague complaints of dizziness are quite common in this population and disorders of the vestibular end organ may be difficult to differentiate from unsteadiness related to other disease processes. Sloane points out that elderly patients are particularly difficult to categorize into a single category, since approximately one-half will have more than one cause for their symptom of dizziness. Even if a single likely etiology has been diagnosed and treated [for example, benign paroxysmal positional vertigo (BPPV)] the patient may remain symptomatic due to another etiology. Orthostatic Hypotension Orthostatic hypotension is a particularly common cause of non-vestibular dizziness in the elderly. The venous system in healthy adults contains a ‘‘reservoir’’ of 0.3–0.8 L of blood. When additional volume is required to maintain perfusion pressure, blood from this pool is added to the circulating volume by muscle contraction. Minimal changes in arterial pressure, primarily sensed by baroreceptors in the aorta and carotid arteries, increase sympathetic tone to maintain cerebral perfusion regardless of body position. The regulatory adjustments occur so rapidly in the young healthy individual that they cannot be measured without the use of invasive instantaneous monitoring. A variety of factors can lead to significant slowing of this response in the elderly. The resultant temporary reduction in cerebral perfusion results in a number of symptoms such as light-headedness, true vertigo, blurring of vision or other visual changes, tremulousness, palpitations, cognitive dysfunction, or frank loss of consciousness. Symptoms are typically temporally related to movement to the upright position from a supine or sitting position. In the aggregate, these symptoms can also be described as ‘‘presyncopal,’’ even in the absence of subsequent syncope. Underlying factors may be cardiac dysfunction, lack of vascular elasticity, reduced muscle tone, neurologic disorders, particularly involving the autonomic nervous system, and volume depletion. Although volume loss may be due to acute blood loss (such as from a gastrointestinal bleed), in the elderly patient it is more typically due to diuretic therapy. In most instances, more than one factor is responsible for the fluctuation in perfusion pressure. For example, patients may be on a beta-blocker and a diuretic. The age-related changes of reduced muscle tone as well as possibly a mild peripheral neuropathy further aggravate the loss of vascular tone. Although each of these factors may be tolerated individually, in the aggregate they combine to create sufficient aberration in blood pressure homeostasis to result in reduced cerebral blood flow and symptoms of dizziness. The most common underlying cause in the elderly is iatrogenic—due to medications, usually antihypertensives (5). Blood volume is reduced by diuretics, blood

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vessel reactivity is reduced by alpha-blockers, and cardiac responsiveness affected by beta-blockers and other medications. Sildenafil, nitrates, and other vasodilating medications can reduce blood pressure (BP) responsiveness and lead to orthostatic hypotension. Bradley and Davis (7) suggest that diagnosis is most easily verified by cautiously eliminating the suspected drug to determine whether it is the cause of the symptoms. On the other hand, the benefit of the drug may warrant its continuation, with care taken on the part of the patient to compensate for the anticipated light-headedness upon arising from a supine position. Vertebrobasilar Insufficiency Defective posterior fossa perfusion due to vascular disease of the basilar artery, one or both vertebral arteries, and inadequate collateral flow from the carotid system is termed vertebrobasilar insufficiency. Unfortunately, vertebrobasilar insufficiency can present with vertigo as the only symptom (4); therefore, the absence of other neurologic symptoms does not rule it out. Vertebrobasilar insufficiency is, in fact, a vestibular disorder, regardless of whether it is due to brainstem or peripheral end-organ ischemia. Evaluation requires vascular imaging, typically initially with a magnetic resonance angiogram (MRA). Current treatment algorithms are in flux due to the recent introduction of endovascular procedures designed to enhance cerebral blood flow, even in the posterior circulation. A particular—and rare—form of vertebrobasilar insufficiency is subclavian steal syndrome, which occurs as a sequela of proximal subclavian artery stenosis. The distal subclavian artery is reconstituted by retrograde flow through the ipsilateral vertebral artery. This condition can be suspected by a history of dizziness brought on by arm exertion, with the diagnosis further supported by differential arm blood pressures. Trans-cervical Doppler study of the vertebral arteries may demonstrate retrograde flow, confirming the diagnosis.

ROLE OF THE OTOLARYNGOLOGIST IN NON-VESTIBULAR DIZZINESS The most common setting in which an otolaryngologist is consulted to evaluate a patient with non-vestibular dizziness is due to the presumptive diagnosis of a vestibulopathy by the referring physician. The otolaryngologist may tend to assume that non-vestibular causes of dizziness have been already considered and ruled out, and that the patient therefore must be suffering from symptoms of a vestibular end-organ disorder. It is best that the consultant begin the evaluation by first assuming that no cause has been ruled out. The symptoms of dizziness and the patient’s past medical history should be reviewed, especially any history of prior cardiovascular illnesses and current medications. Changes in medications, particularly antihypertensive medication, may provide specific clues to the diagnosis of orthostatic hypotension. Careful inquiry to the nature, duration, character, and timing of the episodes may provide important information. ‘‘Are you dizzy in bed before you get up, or after you stand up?’’ is a useful question that can effectively drive further questioning. An appropriate physical examination must be performed, with attention to other possible etiologies of the patient’s symptoms as well as the common vestibulopathies. Finally, when evaluating patients with dizziness the otolaryngologist must assume the role of a general physician and assess other

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systems as well as the vestibular system. The emphasis in this setting should be on identifying possibly life-threatening processes, and rapidly referring the patient for appropriate management. If the patient is found to have a benign balance disorder, then management should be toward reducing the risk of falling, increasing activity levels, and improving the quality of life, usually via an exercise program including vestibular rehabilitation. Management of the dizzy elderly patient is perhaps one of the most challenging problems faced by the primary care physician; hence, there is often a tendency to refer the elderly dizzy patient early in the diagnostic process. The knowledge and skills that are necessary for the diagnosis are not difficult, but in the busy primary care practice the assumption is often made that all dizziness is due to vestibular end-organ disease. The logical next step then is to abbreviate the evaluation and refer without having completed a careful search for a non-vestibular etiology. Empiric treatment with vestibular suppressants is often initiated before the patient is referred for otolaryngologic evaluation. All too often, the patient arrives for the consultation, already on vestibular suppressants, and still complaining of dizziness. The otolaryngologist should not only completely evaluate these patients, but should also use the referral as a teaching case for the primary care physician. Although formal teaching of the management of dizziness via lectures, books, web programs, etc. is useful, a discussion of the specific patient via a phone call may be the single most effective teaching modality for the primary care physician, and assist many more patients.

APPROACH TO THE PATIENT WITH NON-VESTIBULAR DIZZINESS The patient with non-vestibular dizziness presents to the otolaryngologist with generic ‘‘dizziness and giddiness, not otherwise specified’’; hence, the history will be exactly the same for dizziness whether of vestibular or non-vestibular causes. Orthostatic hypotension is a common source of dizziness in the elderly, and may be detected by questions regarding the timing of the onset: ‘‘Did it occur when you rolled over before arising, or shortly after standing up?’’ Occurrence of symptoms during specific head motion may suggest benign paroxysmal postural vertigo, but also may indicate vertebral-basilar insufficiency. Dizziness that accompanies upper extremity exercise may suggest subclavian steal. Associated neurologic symptoms such as syncope (‘‘have you ever fainted or felt like you were going to faint?’’), muscle weakness, tremor, or parathesias may provide an indication that the patients have some other cause of their complaint. A review of cardiac diseases, prior treatment, arrhythmias, medications, recent changes in medications, etc. may point to a cardiac cause. Questioning regarding the use of vestibular suppressants may assist in predicting the accuracy of a Dix-Hallpike or other vestibular testing. A wide variety of causes of dizziness may appear to respond to vestibular suppression, so a history of resolution following initiation of suppressant medication should not be perceived as prima facie evidence of vestibulopathy. Geriatric patients, in particular, may present with dizziness of more than one cause; hence, it is probably safest to assume that any patient could have both a vestibular as well as a non-vestibular cause. The physical examination of any dizzy patient should therefore include a head and neck examination, and a basic cardiovascular examination to include an assessment of the cardiac rate and rhythm, and the status of the carotid arteries, as well as the BP. Pulse and BP should be obtained in both the supine as well as upright positions, especially in the elderly dizzy patient.

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A drop in systolic blood pressure by 20 mm of mercury, or diastolic by 10, is sufficient for the diagnosis of orthostatic hypotension. The cardiac rate should increase to compensate; however, many elderly patients are on cardiac medications or a pacemaker that control cardiac rate, so a compensatory change in rate may not be observed. A focused neurologic examination to include observation of gait, sensory and motor function, particularly of the lower extremities, and assessment of cerebellar function is required. Observation of gaze function, as well as testing for positional nystagmus should be performed. Recall that if the patient is already on vestibular suppression that the characteristic symptoms and physical findings of a Dix-Hallpike maneuver may be absent. Occasionally, patients with vertebrobasilar insufficiency may complain of symptoms reproduced by head position changes, making differentiation from BPPV challenging. The presence of other neurologic symptoms such as visual changes, perioral numbness, etc. suggests that the process is not BPPV and warrants careful neurologic examination and brain imaging. If the history suggests BPPV, even in the absence of positional changes, some physicians routinely perform otolith repositioning in the hope that some benefit may ensue. Audiometry is considered standard in all cases of dizziness, and should be performed even if the initial impression is one of non-vestibular dizziness. Vestibular testing to include posturography is warranted in cases in which the history suggests a balance disorder but the diagnosis is unclear. Imaging is more likely to be required in the elderly dizzy patient than in younger patients, since the diagnosis is frequently less clear and may be confounded by a number of etiologies. In an elderly patient, the acute onset of severe vertigo should be considered to possibly represent a possible posterior fossa vascular event rather than vestibular neuronitis. Hence, urgent referral to an emergency department for evaluation and imaging is required in this population (8).

TREATMENT OF NON-VESTIBULAR DIZZINESS Treatment of non-vestibular dizziness should first address the cause, if identified. In patients with orthostatic hypotension, this will mandate changes in medications, particularly antihypertensives and diuretics. The otolaryngologist will wish to refer the patient back to their primary care physician for adjustments in their medications. It is critical that patients be advised not to stop or reduce their antihypertensive medications without consulting their primary care physicians. The patient should be counseled that the risk of stroke or other disease due to discontinuation of their antihypertensive medications far outweigh any benefits in balance function they might appreciate. Discontinuation of vestibular suppression should be an integral component of management in any elderly patient with non-vestibular (as well as vestibular) dizziness. Patients may be on several vestibular suppressants, including medication administered for management of dizziness as well as anxiolytic medications. Convincing the patient to reduce or discontinue these medications is often the most difficult component of treatment. Treatment with a formal program of vestibular rehabilitation has been shown to be effective in a wide variety of balance disorders, whether or not they originate within the vestibular end organ. Hence, referral for vestibular rehabilitation should be part of any treatment plan for the dizzy elderly patient, regardless of etiology (with the possible exception of isolated BPPV). The details of this treatment modality

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are covered in the subsequent chapters in this text. Evidence suggests that an exercise program, whether or not it is specifically balance-oriented, will provide subjective and objective benefits for balance function as well (9). Patients should be encouraged to engage in a regular exercise program, preferably under the guidance of their primary care physician if they have been sedentary.

SUMMARY Dizziness is common in the elderly, and more than one-half of affected patients suffer dizziness from non-vestibular causes. A taxonomy of possible etiologies assists in determining the most likely cause, but nearly one-half of the dizzy elderly patients will have more than one cause for their symptoms. Orthostatic hypotension due to loss of blood pressure responsiveness is one of the most common causes, and best managed by judicious adjustment of their antihypertensive regimen by the patient’s primary physician. All patients should undergo a careful history, and physical examination with attention to the cardiovascular system, as well as a focused neurologic examination. BPPV should be considered, even in the presence of confusing symptoms, since the treatment of this condition with otolith repositioning may be gratifying. Further testing is required in most patients, with urgent imaging mandated in acute cases to rule out a posterior fossa vascular event. Treatment should focus on identified etiologic processes, discontinuation of vestibular suppressive medication, and formal vestibular rehabilitation. An age-appropriate exercise program will reduce the burden of the disorder in the majority of elderly patients, regardless of the underlying etiology. Finally, discussion of the diagnosis and management with the patient’s referring physician may lead to prompt and appropriate evaluation and management of future elderly patients with dizziness.

REFERENCES 1. Sloane PD, Balzer D, George LK. Dizziness in a community elderly population. JAGS 1989; 37:101–108. 2. Sloane PD, Coeytaux RR, Beck RS, Dallara J. Dizziness: state of the science. Ann Intern Med 2001; 134:823–832. 3. Burker EJ, Wong H, Sloane PD, Mattingly D, Preisser J, Mitchell CM. Fear of falling in dizzy and nondizzy elderly. Psychol Aging 1995; 10:104–110. 4. Tinetti ME, Williams CS, Gill TM. Dizziness among older adults: a possible geriatric syndrome. Ann Intern Med 2000; 132:337–344. 5. Drachman DA. Occam’s razor, geriatric syndromes, and the dizzy patient [editorial]. Ann Intern Med 2000; 132:403–404. 6. Drachman DA, Hart CW. An approach to the dizzy patient. Neurology 1972; 22:323–334. 7. Bradley JG, Davis KA. Am Fam Phys 2003; 68:2393–2398. 8. Furman JM, Cass SP. Vestibular Disorders: A Case-study Approach. New York: Oxford University Press, 2003. 9. Wolfson L, Whipple R, Derby CA, et al. A dynamic posturography study of balance in healthy elderly. Neurology 1992; 42:2069–2075.

13 Benign Paroxysmal Positional Vertigo Joseph M. Furman Division of Balance Disorders, Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

GENERAL DESCRIPTION OF THE CLINICAL PROBLEM Benign paroxysmal positional vertigo (BPPV) is a common neurologic disorder that is particularly common in older individuals (1–3). The etiology of BPPV relates to free-floating otoconial debris in the vestibular labyrinth that causes the semicircular canals, especially the posterior semicircular canal, to become a gravity-sensitive organ (4). Normally, the semicircular canals sense rotational motion but are not sensitive to the orientation of the head with respect to gravity. Conversely, the otolith organs, namely the utricle and the saccule, are gravity sensitive. Patients with BPPV have an inappropriate sensitivity of one or more semicircular canals to the orientation of the head with respect to gravity. Patients with BPPV experience vertigo when they change their head position with respect to gravity. Typical provocative activities include turning in bed and looking up. There are several different varieties of positional vertigo. The most common of these varieties of positional vertigo results from debris floating freely in the posterior semicircular canal. This form of positional vertigo has been called BPPV (5). Other types of positional vertigo can be caused by free-floating debris in the horizontal semicircular canal (6–9) and possibly in the anterior semicircular canal (10,11). Moreover, some forms of positional vertigo are thought to occur as a result of debris attaching itself to the cupula of one of the semicircular canals (12,13). In this circumstance, the debris is adherent rather than free-floating, and causes a variant of BPPV. The incidence of BPPV has been estimated to be between 10 and 100 cases per 100,000 individuals per year (3,14). A recent study has suggested that as many as 9% of geriatric patients have unrecognized BPPV (15). Common antecedents to BPPV include vestibular neuronitis and head trauma (1) but BPPV is often idiopathic especially in older persons. The recurrence rate of BPPV is approximately 1% per month, cumulatively suggesting that about 50% of patients will have a recurrence of BPPV at some time in the first four years following their initial episode (16,17). There are some data to suggest that recurrences of the BPPV are more likely in persons with post-traumatic BPPV. BPPV affects adults of all ages but can be more troublesome in the elderly population for several reasons. Older individuals generally have impaired balance 155

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as compared to younger individuals, even in the absence of a specific balance disorder. Thus, BPPV probably increases the risk for falls and possibly injurious falls in the older population. Moreover, older individuals often have difficulty with other sensations that are important for balance, that is vision and somatosensation. Thus, BPPV may occur as part of a multisensory problem and thereby causes more of impairment with balance in an older individual than it would in a younger one. Also, recovery from BPPV in younger individuals often requires only a particle-repositioning maneuver (to be discussed below). However, older individuals may require vestibular rehabilitation therapy to more fully regain their balance function.

PATHOPHYSIOLOGY OF BPPV It is now well established that BPPV is usually caused by displaced otoconial debris from the utricular macula that is floating freely in the posterior semicircular canal (Fig. 1). As a result, when patients with BPPV change their head position with respect to gravity, e.g., by looking up or rolling over in bed, the mobile otoconial debris moves inside the labyrinth. Movement of the debris presumably causes cupular motion and an associated stimulation of the labyrinth. Patients with BPPV experience spinning vertigo for a brief time after provocative head movements. If the head is kept in a new position, the debris reaches a new stable position and after a few seconds, the vertigo stops. Patients may, however, feel a sense of nausea and disequilibrium even after the vertigo stops. Also, patients with BPPV, especially older individuals, often experience imbalance, probably as a result of a malfunctioning labyrinth or central nervous system suppression of vestibular signals. Older

Figure 1 Free-floating endolymph particles within the posterior semicircular canal observed during surgery. The posterior semicircular canal had been opened in preparation for a canal plugging procedure. White debris was observed within the endolymph compartment of the posterior semicircular canal. These two photographs are sequential in time and show that the particles shifted during movement of the patient’s head; compare shape and position of particles at straight arrow with the curved arrow. Source: From Ref. 18.

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patients who have been placed on a vestibular suppressant medication for BPPV may have a worsening of their balance as a secondary effect of their medications, in addition to the underlying BPPV. There are atypical forms of BPPV. In a small percentage of patients, free-floating debris apparently affects the horizontal semicircular canal rather than the posterior semicircular canal (6–9). Involvement of the anterior semicircular canal has been postulated but remains controversial (10,11). These atypical forms of BPPV also cause paroxysmal, that is, brief, episodes of positional vertigo. In some patients, debris apparently becomes adherent to the cupula of one of the semicircular canals and is thus fixed rather than free-floating (12,13). In these patients, vertiginous symptoms are typically not paroxysmal but rather persist for as long as the patient maintains the head tilted position.

CLINICAL PRESENTATION Most patients with BPPV provide a clear history of vertiginous episodes during activities of looking up and turning in bed. In fact, without such a history, BPPV is unlikely. Many patients describe periods of time lasting from days to weeks during which they have positional symptoms but note having symptom-free intervals as well. Thus, many patients with a history characteristic for BPPV may not be suffering from the condition at the time of evaluation. Also, some patients relate experiencing an acute vestibular syndrome prior to the onset of symptoms typical for BPPV. Conditions such as vestibular neuritis, labyrinthine concussion (1), and possibly migraine-related vestibulopathy (19,20) may precede the onset of BPPV, but BPPV often occurs spontaneously. Vertiginous symptoms that occur without provocation, e.g., vertigo when someone is sitting still, argue against BPPV; in this case, other etiologies should be considered, e.g., Meniere’s disease. Moreover, patients with auditory complaints or neurologic complaints other than dizziness or imbalance should be evaluated for conditions other than BPPV. The diagnosis of BPPV can be confirmed using the Dix-Hallpike maneuver (Fig. 2). This maneuver is most helpful for diagnosing typical BPPV, i.e., free-floating particles in the posterior semicircular canal. Performing the Dix-Hallpike maneuver can be more challenging in older individuals as a result of decreased range of motion of the neck. In such patients, a modified Dix-Hallpike maneuver may be required in which the patient’s head is not extended over the edge of the examining table. Observing the patient’s eye movements using Frenzel’s glasses or infrared video-goggles rather than simply in a lighted room can increase the diagnostic accuracy of the Dix-Hallpike maneuver. Patients with BPPV will exhibit a nystagmus with a characteristic latency of onset, duration, and direction (Fig. 2; Table 1). The nystagmus of BPPV is typically accompanied by subjective vertigo. Any deviation from these characteristic findings should raise a concern that an alternative diagnosis may be present. Especially worrisome is downbeating nystagmus in the ear-down position as this may suggest a posterior fossa abnormality. In patients with negative Dix-Hallpike maneuver, an evaluation for lateral (horizontal) semicircular canal BPPV should be performed using the ‘‘roll test.’’ The ‘‘roll test’’ actually uses yaw head movement and is performed by turning the patient from the supine to the head left or head right position and then to the opposite, i.e., head right or head left, position. Patients with lateral semicircular canal BPPV will experience vertigo after turning their head both to the left and to the right. Horizontal nystagmus beating toward the down ear will be observed while

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Figure 2 The Dix-Hallpike test of a patient with BPPV affecting the right ear. In panel (A), the examiner stands at the patient’s right side and rotates the patient’s head 45 to the right to align the right posterior semicircular canal with the sagittal plane of the body. In panel (B), the examiner moves the patient, whose eyes are open, from the seated to the supine right-ear-down position and then extends the patient’s neck slightly so that the chin is pointed slightly upward. The latency, duration, and direction of nystagmus, if present, and the latency and duration of vertigo, if present, should be noted. The arrows in the inset depict the direction of nystagmus in patients with typical BPPV. The presumed location in the labyrinth of the free-floating debris thought to cause the disorder is also shown. Abbreviation: BPPV, benign paroxysmal positional vertigo. Source: From Ref. 16. Table 1 Characteristics of Benign Paroxysmal Positional Vertigo      

Positionally induced vertigo and nystagmus using the Dix-Hallpike maneuver Latency of 1–40 sec (average 3–4 sec) Nystagmus is torsional—vertical Duration of signs and symptoms 10–60 sec Decreased signs and symptoms with repeated positioning Responds to particle repositioning

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the patient is vertiginous. The vertigo and nystagmus is often severe, being worse when the patient is turned toward the involved ear. In the geriatric population, Dix-Hallpike maneuver may be confounded by the presence of other medical illnesses, especially orthostatic intolerance, which may lead to a sense of dizziness when the patient is moved from a recumbent to a sitting position. In these circumstances, patients typically do not report vertigo; rather, they report lightheadedness. Patients with BPPV provide such a characteristic history and have such a characteristic physical examination in that no other disorder presents precisely in the same way. However, in some patients, the historical information is unclear and the Dix-Hallpike maneuver may be equivocal. Alternative diagnoses other than BPPV that should be considered as a cause of episodic vertigo include endolymphatic hydrops, i.e., Meniere’s disease, vertebro-basilar insufficiency, migraine, and anxiety, particularly panic attacks. Usually, one or more aspects of the patient’s presentation will allow differentiation between BPPV and one of these other disorders. However, there can be diagnostic uncertainty, especially in patients who are suffering from more than one condition. Vestibular laboratory testing is not required for the diagnosis of BPPV. However, vestibular laboratory testing can provide information regarding whether the BPPV is an isolated problem or is an indication of more widespread vestibular disease. A unilateral caloric reduction suggests that the horizontal semicircular canal is involved in the patient’s disorder. A directional preponderance on rotational testing or a spontaneous vestibular nystagmus suggests an ongoing vestibulo-ocular reflex asymmetry. Platform posturography testing may provide information regarding how a patient is using vestibular information for upright balance. Thus, although vestibular laboratory testing is not required for arriving at a diagnosis of BPPV, the tests are noninvasive and provide information that can overall be helpful in managing a patient’s symptoms of dizziness and imbalance. Vestibular laboratory testing can be especially helpful for (i) deciding whether or not an individual should be referred for vestibular rehabilitation therapy, (ii) advising the patient regarding their activities, either at work or at home, and (iii) helping to prognosticate as to the length of time that will be required for recovery.

TREATMENT OF BPPV The treatment of BPPV consists of repositioning the displaced otoconial debris from the posterior semicircular canal back to the region of the utricle where it no longer provokes symptoms. The particle-repositioning maneuver for patients with posterior canal BPPV, illustrated in Figure 3 (21), is highly successful, with a reported rate of success of approximately 90% (16,22,23). Performing this maneuver in elderly individuals is usually quite straightforward and can be performed without risk. An alternate particle-repositioning maneuver for patients with posterior canal BPPV, the so-called ‘‘liberatory maneuver’’, requires abrupt movements that are inappropriate for the elderly population (24). Since the liberatory maneuver has not been shown to be more successful than the particle-repositioning maneuver described in Figure 3, we do not advocate it. Following particle repositioning, many clinicians suggest that patients remain upright for 24 to 48 hours so that the otoconial debris is more likely to stay out of the posterior semicircular canal (16,21). However, remaining upright following particle repositioning is not recommended by all experts and has not been shown convincingly to alter treatment outcome (25,26). Thus, especially in older

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Figure 3 (Caption on facing page) Figure 3 (Caption on facing page)

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Figure 4 Brandt–Daroff exercises for benign paroxysmal positional vertigo. Source: From Ref. 31.

patients, recommending that the patient remain upright for 24 to 48 hours may not be appropriate. For lateral canal BPPV, there is a different kind of particle-repositioning maneuver that should be performed (27,28). A small percentage of patients with BPPV do not respond to particle repositioning initially and need to undergo a repeat particle-repositioning maneuver. Some patients cannot undergo particle repositioning either because of musculoskeletal problems, immobility, apprehension, or severe nausea at the time of assessment. For these individuals, Brandt–Daroff exercises (29,30), illustrated in Figure 4, should be prescribed. Rarely, patients with BPPV cannot be treated successfully with particle repositioning or Brandt–Daroff exercises or suffers from frequent recurrences. In these rare individuals, surgical

Figure 3 (Facing page) Bedside maneuver for the treatment of a patient with BPPV affecting the right ear. The presumed position of the debris within the labyrinth during the maneuver is shown in each panel. The maneuver is a three-step procedure. First, a Dix-Hallpike test is performed with the patient’s head rotated 45 toward the right ear and the neck slightly extended with the chin pointed slightly upward. This position results in the patient’s head hanging to the right (panel A). Once the vertigo and nystagmus provoked by the Dix-Hallpike test cease, the patient’s head is rotated about the rostral–caudal body axis until the left ear is down (panel B). Then the head and body are further rotated until the head is face down (panel C). The vertex of the head is kept tilted downward throughout the rotation. The maneuver usually provokes brief vertigo. The patient should be kept in the final, face-down position for about 10 to 15 seconds. With the head kept turned toward the left shoulder, the patient is brought to the seated position (panel D). Once the patient is upright, the head is tilted so that the chin is pointed slightly downward. Abbreviation: BPPV, benign paroxysmal positional vertigo. Source: From Ref. 16.

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Figure 5 Algorithm for managing patients with benign paroxysmal positional vertigo.

intervention may be indicated. Posterior semicircular canal occlusion appears to be the surgical procedure of choice for such patients (32). However, advanced age is a relative contraindication for this elective surgery. Figure 5 provides an algorithm for managing patients presenting with positional vertigo. Note that many older patients with BPPV, even if treated successfully for their positional vertigo, will benefit from vestibular rehabilitation therapy.

REFERENCES 1. Baloh RW, Honrubia V, Jacobson K. Benign positional vertigo: clinical and oculographic features in 240 cases. Neurology 1987; 37(3):371–378. 2. Bourgeois PM, Dehaene I. Benign paroxysmal positional vertigo (Bppv). Clinical features in 34 cases and review of literature. Acta Neurol Belg 1988; 88(2):65–74. 3. Mizukoshi K, Watanabe Y, Shojaku H, Okubo J, Watanabe I. Epidemiological studies on benign paroxysmal positional vertigo in Japan. Acta Otolaryngol Suppl 1988; 447:67–72. 4. Hall SF, Ruby RR, McClure JA. The mechanics of benign paroxysmal vertigo. J Otolaryngol 1979; 8(2):151–158. 5. Lanska DJ, Remler B. Benign paroxysmal positioning vertigo: classic descriptions, origins of the provocative positioning technique, and conceptual developments. Neurology 1997; 48(5):1167–1177. 6. Baloh RW, Jacobson K, Honrubia V. Horizontal semicircular canal variant of benign positional vertigo. Neurology 1993; 43(12):2542–2549.

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7. De la Meilleure G, Dehaene I, Depondt M, Damman W, Crevits L, Vanhooren G. Benign paroxysmal positional vertigo of the horizontal canal. J Neurol Neurosurg Psychiat 1996; 60(1):68–71. 8. Lempert T. Horizontal benign positional vertigo. Neurology 1994; 44(11):2213–2214. 9. Pagnini P, Nuti D, Vannucchi P. Benign paroxysmal vertigo of the horizontal canal. J Otorhinolaryngol Relat Spec 1989; 51(3):161–170. 10. Herdman SJ, Tusa RJ. Complications of the canalith repositioning procedure. Arch Otolaryngol Head Neck Surg 1996; 122(3):281–286. 11. Honrubia V, Baloh RW, Harris MR, Jacobson KM. Paroxysmal positional vertigo syndrome. Am J Otolaryngol 1999; 20(4):465–470. 12. Baloh RW, Yue Q, Jacobson KM, Honrubia V. Persistent direction-changing positional nystagmus: another variant of benign positional nystagmus? Neurology 1995; 45(7):1297–1301. 13. Schuknecht HF. Cupulolithiasis. Arch Otolaryngol 1969; 90(6):765–778. 14. Froehling DA, Silverstein MD, Mohr DN, Beatty CW, Offord KP, Ballard DJ. Benign positional vertigo: incidence and prognosis in a population-based study in Olmsted County, Minnesota. Mayo Clin Proc 1991; 66(6):596–601. 15. Oghalai JS, Manolidis S, Barth JL, Stewart MG, Jenkins HA. Unrecognized benign paroxysmal positional vertigo in elderly patients. Otolaryngol Head Neck Surg 2000; 122(5):630–634. 16. Furman JM, Cass SP. Benign paroxysmal positional vertigo. N Engl J Med 1999; 341(21):1590–1596. 17. Hain TC, Helminski JO, Reis IL, Uddin MK. Vibration does not improve results of the canalith repositioning procedure. Arch Otolaryngol Head Neck Surg 2000; 126(5):617–622. 18. Parnes LS, McClure JA. Free-floating endolymph particles: a new operative finding during posterior semicircular canal occlusion. Laryngoscope 1992; 102(9):988–992. 19. Ishiyama A, Jacobson KM, Baloh RW. Migraine and benign positional vertigo. Ann Otol Rhinol Laryngol 2000; 109(4):377–380. 20. Lempert T, Leopold M, von Brevern M, Neuhauser H. Migraine and benign positional vertigo. Ann Otol Rhinol Laryngol 2000; 109(12 Pt 1):1176. 21. Epley JM. The canalith repositioning procedure: for treatment of benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1992; 107(3):399–404. 22. Lynn S, Pool A, Rose D, Brey R, Suman V. Randomized trial of the canalith repositioning procedure. Otolaryngol Head Neck Surg 1995; 113(6):712–720. 23. Steenerson RL, Cronin GW. Comparison of the canalith repositioning procedure and vestibular habituation training in forty patients with benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1996; 114(1):61–64. 24. Semont A, Freyss G, Vitte E. Curing the Bppv with a liberatory maneuver. Adv Otorhinolaryngol 1988; 42:290–293. 25. Massoud EA, Ireland DJ. Post-treatment instructions in the nonsurgical management of benign paroxysmal positional vertigo. J Otolaryngol 1996; 25(2):121–125. 26. Nuti D, Nati C, Passali D. Treatment of benign paroxysmal positional vertigo: no need for postmaneuver restrictions. Otolaryngol Head Neck Surg 2000; 122(3):440–444. 27. Epley JM. Positional vertigo related to semicircular canalithiasis. Otolaryngol Head Neck Surg 1995; 112(1):154–161. 28. Nuti D, Agus G, Barbieri MT, Passali D. The management of horizontal-canal paroxysmal positional vertigo. Acta Otolaryngol 1998; 118(4):455–460. 29. Brandt T, Daroff RB. Physical therapy for benign paroxysmal positional vertigo. Arch Otolaryngol 1980; 106(8):484–485. 30. Brandt T, Steddin S, Daroff RB. Therapy for benign paroxysmal positioning vertigo, revisited. Neurology 1994; 44(5):796–800. 31. Herdman SJ. Assessment and management of benign paroxysmal positional vertigo. In: Herdman SJ, ed. Vestibular Rehabilitation. Philadelphia: FA Davis, 1999:20.

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32. Parnes LS, McClure JA. Posterior semicircular canal occlusion in the normal hearing ear. Otolaryngol Head Neck Surg 1991; 104(1):52–57.

ANNOTATED BIBLIOGRAPHY Furman JM, Cass SP. Benign paroxysmal positional vertigo. N Engl J Med 1999; 341(21):1590–1596. An excellent review of benign paroxysmal positional vertigo. Lanska DJ, Remler B. Benign paroxysmal positioning vertigo: classic descriptions, origins of the provocative positioning technique, and conceptual developments. Neurology 1997; 48(5):1167–1177. A detailed account of the development of ideas concerning the pathogenesis, diagnosis, and treatment of benign paroxysmal positional vertigo. Issues regarding nomenclature are discussed. Lynn S, Pool A, Rose D, Brey R, Suman V. Randomized trial of the canalith repositioning procedure. Otolaryngol Head Neck Surg 1995; 113(6):712–720. A well-written account of particle repositioning for benign paroxysmal positional vertigo. Parnes LS, Agrawal SK, Atlas J. Diagnosis and management of benign paroxysmal positional vertigo (BPPV). Can Med Assoc J 2003; 169(7):1–16. Another excellent review of benign paroxysmal position vertigo.

14 Balance Rehabilitation Horst R. Konrad Division of Otolaryngology—Head and Neck Surgery, Southern Illinois University School of Medicine, Springfield, Illinois, U.S.A.

Meiho Nakayama Department of Otolaryngology, Aichi Medical University, Aichigun Aichiken, Japan

Marian Girardi—In Memoriam I had the privilege of working with Dr. Marian Girardi our former coauthor from November 1, 1993 to June 30, 2003. Dr. Girardi first joined our vestibular laboratory at a time when vestibular testing and balance rehabilitation was in its infancy. In the Southern Illinois University Vestibular Laboratory, she established normal values for elderly patients by testing many patients from the senior citizens groups in the Springfield area. These values are still used in most clinics in the evaluation and treatment of elderly patients with balance disorders. Dr. Girardi’s academic life centered around the evaluation and management of patients with balance disorders, especially elderly patients. This subject also became her doctoral thesis. She taught extensively at SIU School of Medicine. She established and directed our first balance disorders and falls prevention clinic. She lectured extensively to medical students, residents, and faculty and developed a balance assessment and management team, which included otolaryngologists, audiologists, physical and occupational therapists, and fellows and residents from otolaryngology, neurology, and other services in the medical school. She also lectured extensively both nationally and internationally, and she was a pioneer in recognizing the usefulness of posturography and patient questionnaires to evaluate balance function and its impact on quality of life. She also published extensively in the medical literature on the subject of balance evaluation and treatment. In addition to her academic achievements, she was very heavily involved in the 4-H Clubs of central and southern Illinois, where she taught horseback riding, dressage, cooking, baking, and many other 4-H activities. She died on December 5, 2004, of complications of diabetes. She will be sorely missed by her colleagues, her students, and her many friends in our community.

Horst R. Konrad M.D.

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INTRODUCTION Balance rehabilitation relies on innate restorative mechanisms within the brain and the body to re-establish function lost through disease, injury, medication, surgical ablation, or disuse. Balance function requires: (i) sensory information from vision, vestibular, and proprioceptive systems, (ii) integration of this information by the central nervous system, and (iii) execution of motor activities by the musculoskeletal system. Many of these activities occur through reflexive action such as the spinal reflexes, the vestibulo-oculomotor reflexes, the visual oculomotor reflexes, and the vestibulospinal reflexes. Cerebral function controls and alters these reflexes while cerebellar activity ‘‘fine tunes’’ and adjusts the responses. Many of these systems are well known. The vestibulo-oculomotor reflex is one of the best examples. Early work in rehabilitation is credited to Cooksey and Cawthorne in England in the 1940s. Cooksey, a physical therapist, and Cawthorne, an otolaryngologist, worked together to help patients with vestibular deficits improve their function through a physical therapy program. Later Brant Daroff exercises were popularized to help patients with balance impairment. Work by Makoto Igarashi et al. (1) at Baylor showed that the physiological basis of vestibular rehabilitation were dependent on head movement during vision. Experiments with cats and monkeys showed that animals with vestibular lesions did not compensate well when in the dark, compensated better when kept in the light and left to exercise at liberty, and improved even faster when forced to exercise in light. Igarashi et al. also showed that the neural elements for this improvement were the vestibular nuclei and the cerebellum where information from the visual system and the vestibular system could be utilized by cerebellar neurons to ‘‘reset’’ the sensitivity and gain of the vestibulo-ocular motor reflex. This improved understanding of the mechanisms involved in the resetting of the vestibulo-ocular motor reflex has been incorporated into exercise programs for patients who have uncompensated or inadequately compensated peripheral vestibular deficits. Most patients with balance disorders benefit from a rehabilitation program. The magnitude of the benefit depends on many factors, including the characteristics of the disorder, age, and motivation. Evaluation of the patient’s deficits as well as remaining function is critical in providing an efficient and effective rehabilitation program for the patients. Patients with stable, purely vestibular deficits have a high success rate with rehabilitation. Patients with a combination of peripheral sensory and central disorders or multisensory and central disorders are less likely to have full resolution of their problems, but are still likely to benefit from rehabilitation. These patients can particularly benefit by a reduction of falls and falls-related injuries. Rehabilitation includes the use and training with assistive devices such as canes and walkers, as well as an assessment and changes in the home. Often changes in lighting and floor surfaces, provision of handholds on steps and bathrooms, and an educational program for the patient’s family and caregivers will lead to significant benefits. Benign paroxysmal positional vertigo (BPPV) is a peripheral vestibular but paradoxical (unstable) disorder, which is very amenable to rehabilitation. It represents an excellent demonstration of the axiom that knowledge of the pathophysiology leads to treatment paradigms with very high success rates. It is probably the most common vestibular disorder affecting balance and is increasingly common in older populations. BPPV was first described by Barany in 1921 (2) and further defined by Dix and Hallpike in 1952 (3). Dix and Hallpike described a provocative test maneuver that

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was diagnostic if it elicited torsional nystagmus directed to the undermost ear, with latency, limited duration, reversal upon reversing the maneuver, and response decline with repetition. In 1969, Schuknecht (4) documented the pathology of some cases of BPPV to be otolithic debris attached to the cupula of the posterior canal. This theory, cupulolithiasis, was believed as a mechanism of BPPV until other investigator documented cases of BPPV arose with a plug of material within the posterior canal itself, the so-called canalolithiasis, rather than material attached to the cupula (5,6). A paucity of temporal bone pathology cases with cupulolithiasis in this common disorder further suggests canalolithiasis as the probable cause of BPPV. It has since become apparent that the mechanism for BPPV is canalolithiasis, which are free-floating debris (or densities) collected within the posterior semicircular canal of the involved ear. This debris shifts in response to gravitational change, creating drag on the perilymph that is then imparted to the cupula. This triggers a nonphysiologic vestibulo-ocular reflex of the posterior semicircular canal, producing nystagmus in its plane. When the debris reach the limit of its descent, the cupula resumes its normal state and the nystagmus terminates. On reversal of the maneuver, the motion of the debris reverses, causing nystagmus with the same characteristics, but in the opposite direction. During the nystagmus period, patients complain of severe dizziness. In 1992, Epley (7) described the canalith repositioning procedure of a fiveposition cycle of head movements. Epley postulated that the maneuver caused free canaliths to migrate by gravity out of the posterior semicircular canal to the vestibule, where they would no longer exert a rotational response. A high success rate was reported and the technique has become accepted by many as the first line of management of BPPV. This describes most cases of BPPV. It is possible, although less common, for the debris to be in an anterior vertical, or the horizontal canal. In these cases, the positional nystagmus will be in the planes of these canals and can be treated by a maneuver in the planes of these canals. It is also possible for patients to have debris in more than one canal or for debris to be moved from one canal to another by the maneuver. For this reason, it is important to re-examine patients in two or more weeks after the performance of a maneuver. This is best done with the use of infrared goggles, which block the fixation reflex and allow the observer to see the eyes and their movements. In-depth discussion of BPPV can be found in the chapter in this text by Joseph Furman. Furthermore, it has been demonstrated that an exercise program following successful resolution of BPPV via the particle-repositioning maneuver reduces the recurrence of this condition (8).

GENERAL DESCRIPTION OF THE CLINICAL PROBLEM Prevalence Balance disorders are extremely common in all populations, accounting for 42% of physician visits. Approximately six million Americans annually seek medical help for dizziness or balance disorders. This is a particularly important issue in geriatrics since, as the populations age, balance disorders become increasingly common. For patients over age 65, this becomes the most common reason for visiting a physician, and even after that, balance problems continue to increase with advancing age. Balance disorders in the aging population not only lead to discomfort and limit the patient’s activities, but also lead to falls and falls with injuries. In addition to this,

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the fear of falling associated with balance disorders severely affects the patient’s quality of life. Fear of falling results in further limitation of movement, which, in turn, results in further weakness, stiffness, and a higher risk of falling with injury. In addition to changes in the patients’ quality of life, balance disorders also affect other members of the family, since as these patients become progressively more incapacitated, children, spouses, or other family members and caregivers must assume roles in activities of daily living, including shopping, preparation of food, and even assisting in walking within the living area from bedroom to bathroom. Older populations, particularly women, are the most rapidly growing parts of the population in the United States and other developed countries. By 2010, more than 40 million Americans will be older than age 65. Both depression and anxiety states are common in patients with balance disorders and further result in limitation of activities. Normal Aging Related to Balance As we age, we lose sensitivity in the vestibular organs—both the rotational sensors, the semicircular canals, and the gravity receptors, the urtricle and saccule. This reduced sensitivity is due to a reduction in numbers of hair cells, particularly in the superior portion of the cupula of the semicircular canals (9). Aging also results in reduction of nerve fibers conducting vestibular information to the brain stem. Other normal changes that affect balance include slowing of central reflexes, particularly in the eye-movement control reflexes and in reflexes required to initiate body movements. Vision and hearing also decline in aging populations, and the function of proprioceptive sensory systems also declines. In addition to this, older patients tend to have less muscle mass and strength, and reduced flexibility and bone strength. Osteoporosis is particularly common in older women and further leads to more severe injuries when a fall occurs. In addition to these normal changes, there are also age-related disorders that significantly affect balance. Benign paroxysmal positional vertigo, described above, is much more common in aging populations. Vascular, carotid artery, vertebral basilar, and small vessel cerebrovascular disease can significantly affect circulation to important brain structures, which are required in eye, head, and limb movements. Transient ischemic attacks and cerebrovascular accidents can further damage brain structures important for balance. Coronary artery disease, cardiomyopathy, and cardiac arrhythmias can also result in decreased brain perfusion, which can add to problems in balance. Many neurological disorders including stroke, degenerative diseases, head injuries, and concussion are more common in the elderly and can lead to balance disorders. Parkinson’s disease as well as degenerative cerebellar and brainstem ataxias also adversely affect balance. Diabetes mellitus reduces vision and proprioceptive function, and also increases arteriosclerosis in both small and large vessels. Hypertension leads to accelerated arteriosclerosis, and treatment leads to postural hypotension or labile blood pressure, which results in orthostatic changes, which can also increase risk of falling. Medications are more commonly used in the elderly populations, and many of the medications currently used can adversely affect balance. Benzodiazepines, such as Valium, and antihistamines such as Meclizine, can reduce vestibular sensitivity and adversely affect balance and recovery from vestibular injury. In younger populations, balance disorders are often characterized by episodes of dizziness or vertiginous episodes, while in the older population, the presenting symptom is generally a feeling of light-headedness, a feeling of disequilibrium or difficulty with walking. Positional vertigo is also a common symptom, and is often the first symptom in benign

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paroxysmal positional vertigo. The physical examination in patients complaining of balance disorders should focus on the sensory systems associated with balance, and also the functions of the cerebellum and cerebrum. Slowing of reflexes is a common cause of balance dysfunction in the elderly. Vision and eye movements need to be carefully assessed, and vision should be corrected when possible. Hearing and balance often go together, so clinical examination of both should be routine. Eye movements, including smooth pursuit, saccade, and observation for spontaneous nystagmus are part of the office examination. Positional tests can easily be done in the office and are very important in patients with symptoms of positional vertigo. Since balance disorders may be due to an intermittent disorder, every opportunity should be utilized to assess this problem at the time symptoms suggest its presence. Proprioception can be examined in the office and as part of the balance evaluation. In addition to this, general strength and flexibility and evaluation for the disorders, which affect balance, are part of the history and physical examination. Electronystagmography, posturography, and audiometry are parts of the laboratory evaluation for these patients. Posturography is the best predictor of falls related to balance problems in the elderly, particularly the test conditions where vision and proprioception are impaired. Another valuable component of posturography testing is the limits of stability test, which determines how far patients can lean before becoming unstable. Electronystagmogram with particular attention to the saccadic latencies, the caloric sensitivity, and visual fixation suppression of caloric nystagmus is helpful in assessing the sensory systems related to inner ear and eye movement control and speed of central processing. Treatment is based on the specific patient’s problem. The symptoms of many patients can be dramatically reduced by eliminating or changing medications that adversely affect balance, cause sedation, or impair reflexes. As a result, medications should be reviewed in all patients who complain of dizziness, particularly the elderly. This strategy is particularly valuable with patients who are on antihistamines, benzodiazepines, or other sedating medications. Detailed description of rehabilitation exercises is beyond the scope of this chapter. Physicians would be advised to work with physical therapists, audiologists, or occupational therapists who are trained in the specific rehabilitation exercise programs. Not all therapists are trained in balance rehabilitation, but in most communities at least one or two therapists are knowledgeable and skilled in the techniques. Patients should be referred to these individuals, and encouraged to learn and practice regularly the techniques that they are taught. Most patients with chronic disequilibrium can be significantly helped with balance rehabilitation. The basis of this benefit is adaptation, compensation, and restoration. In addition to improving strength and flexibility, vestibular balance rehabilitation training attempts to re-establish functions of the vestibular system by utilizing vision and balance exercises. Oculomotor skills can be improved through an exercise program aimed at eye movement control. Posture control can be improved through exercises, and strengthening and flexibility training. Patients are also taught better movement strategies so that the likelihood of falling is diminished. Some of the limitations of balance retraining include loss of vision, proprioception, and loss of vestibular sensitivity. However, balance retraining often can utilize one of these senses to overcome deficiencies in the others. The most difficult problems to manage are severe central nervous system (CNS) problems related to degenerative processes, strokes, or head injuries. Memory, cognitive abilities, and decision making are integral to balance retraining and are adversely affected by such disorders as Alzheimer’s disease, small vessel atherosclerosis, and other degenerative

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CNS disorders. Lack of motivation, anxiety disorder, and other psychological disorders can limit the efficacy of balance retraining and often need to be separately addressed during rehabilitation. Secondary gain and litigation can also be detrimental to good outcomes for balance retraining. It is important to assess not only the patient himself, but also family relationships, the potential for caregivers, and transportation options. Those who are likely to do well for the balance retraining exercises are those with stable vestibular dysfunction, in whom symptoms are persistent, but not progressing at a rapid rate. Poor individuals for vestibular rehabilitation are patients with rapidly progressing disease or with unstable lesions such as frequent attacks of Meniere’s disease or those with demyelinating disorders and seizures. Patients with benign paroxysmal positional vertigo have an outstanding cure rate, in excess of 90%. Patients who have had vestibular neuronitis, labyrinthitis, or postsurgical vestibular damage; postvestibular ototoxicity patients; head trauma patients; and those who have suffered stroke or other forms of central toxicity but who are now stabilized are excellent candidates for rehabilitation. Patients with both central and peripheral disorders, such as head injury patients who received severe brain concussion and lost vestibular function from their head trauma, often suffer from persistent symptoms and may not benefit extensively. Patients are best evaluated and treated through a falls risk assessment and treatment program. The treatment team often includes an otolaryngologist or a neurologist with a special interest in balance disorders. An audiologist, physical therapist, and occupational therapist are integral and equal members of the team. Each one of these individuals brings a special interest, knowledge, and training to benefit patients with balance disorders. Since many of these patients are elderly and have transportation problems, the team is best assembled at one time and placed to avoid separate appointments. Since family and other caregivers are very important for the success of this program, they should accompany the patients and participate in their evaluation and treatment. The use of a questionnaire mailed to the patient that outlines the history, list of medications, and previous treatments and evaluations is often very useful. Audiograms, electronystagmographic studies, imaging, and laboratory studies performed elsewhere are requested, as well as permission to obtain these studies prior to the first appointment. At the first visit, an extensive history and physical examination is performed by the physician. The patient and caregiver or family are then evaluated by the physical therapist, occupational therapist, and audiologist. The treatment team reviews the findings and establishes a plan with specific recommendations for treatment. Additional tests may be necessary, and follow-up must be arranged. The final plan is discussed with the patient and caregivers and sent to the referring clinician. Therapy is instituted and the patient is usually re-evaluated in two to three months. For additional information on patients with multifactorial deficits causing balance dysfunction, see the chapter by Susan L. Whitney, Ph.D., ‘‘Multisensory impairment in older adults: evaluation and intervention.’’

SUMMARY Balance rehabilitation is a critical part of the management of the dizzy elderly patient. Nearly all patients with dizziness from a variety of causes will benefit, as well as those with other balance disorders due to peripheral, central, or musculoskeletal etiologies. Balance rehabilitation therapy should be part of a falls-reduction program

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administered by a multidisciplinary team. Patients with balance disorders should be encouraged to be as active as physically possible in order to maintain and restore balance function. Elimination of sedative medication is often a critical adjunct to a program of balance rehabilitation. Communication and coordination with the patient’s primary care physician is integral to any program of balance rehabilitation.

REFERENCES 1. Igarashi M, et al. Clinical pathological correlations in squirrel monkeys after suppression of semicircular canal function by streptomycin sulfate. Acta Otolaryngol 1966; 214:1–28. 2. Barany R. Diagnose von Krankheitserscheinungen im Bereiche des Otolithenapparates. Acta Otolaryngol 1921; 2:434–437. 3. Dix MR, Hallpike CS. The pathology, symptomatology and diagnosis of certain common disorders of the vestibular system. Ann Otol Rhinol Laryngol 1952; 61:987–1016. 4. Schuknecht HF. Cupulolithiasis. Arch Otolaryngol 1969; 90:765–778. 5. Hall SF. The mechanics of benign paroxysmal vertigo. J Otolaryngol 1979; 8:151–158. 6. Epley JM. New dimensions of benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1980; 88:599–605. 7. Epley JM. The canalith repositioning procedure: for treatment of benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1992; 107:399–404. 8. Amin M, Girardi M, Neill ME, et al. Effects of exercise on prevention of recurrence of BPPV symptoms. ARO Midwinter Meeting, in Feb. 14–18, 1999, St. Petersburg Beach, FL. 9. Nakayama M, Helfert RH, Konrad HR, Caspary DM. Scanning electron microscopic evaluation of age-related changes in the rat vestibular epithelium. Otolaryngol Head Neck Surg 1994; 111:799–806.

PART II: TASTE, SMELL, AND NASAL PROBLEMS

15 Olfaction and Aging Karen J. Fong Department of Otolaryngology—Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A.

Mark A. Zacharek Department of Otolaryngology—Head and Neck Surgery, Henry Ford Hospital, Detroit, Michigan, U.S.A.

INTRODUCTION Olfaction is an important sensory function. Although it is known to decline with age, not all olfactory loss occurring in older adults can be attributed to aging (1–3). Sudden loss in olfaction should be cause for concern in all age groups. Olfactory loss has significant health implications and may increase an elderly individual’s risk of injury or illness from inhalation of gaseous fumes, consumption of rotten food, and deficient caloric intake. In the past decade, much interest has been focused on the changes that occur in the olfactory system during normal aging and the means by which these changes can be measured. This chapter will attempt to review the current understanding of the changes in the olfactory system during aging and the clinical approach to the elderly patient with an olfactory deficit. Based on the National Health Interview Survey, approximately 2.7 million Americans suffer from chronic olfactory problems. The prevalence of this problem was found to be greatest in patients 75 years of age and older (46 per 1000). Adults aged 65–74 years reported the next highest rate at 26.5 per 1000. Taken together, these groups account for 40% of the 2.7 million adults in the United States reporting a chronic problem with their sense of smell (4). Traditionally, determining the prevalence of olfactory sensory changes has relied heavily on ‘‘self-reporting’’ by patients. This approach greatly underestimates the actual incidence of olfactory deficits in the elderly (5). A more recent study in which clinical olfactory testing was used revealed a much higher prevalence of olfactory impairment in older adults than previously identified by self-reporting: in the age groups of 60–69, 70–79, and 80–97, the prevalence rates of olfactory impairment were 17.3%, 29.2%, and 62.5%, respectively (6).

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Table 1 Definition of Terms Relating to Olfactory Dysfunction Anosmia Hyposmia Dysosmia Parosmia Phantosmia Hyperosmia

Complete olfactory loss Diminished sense of smell Distorted olfactory perception In response to an olfactory stimulant In the absence of an olfactory stimulant Enhanced olfactory perception

DEFINITIONS Although multiple terms have been used to describe olfactory dysfunction, the use of standardized terminology is important (Table 1). Anosmia refers to a total absence of olfaction, whereas hyposmia refers to diminished smell sensitivity. Dysosmia refers to a distorted olfactory perception. This can occur in the presence of a sensory stimulus (parosmia) or in the absence of a sensory stimulus (phantosmia). Hyperosmia refers to increased odorant sensitivity. ETIOLOGIES OF OLFACTORY LOSS The etiologies of olfactory dysfunction are numerous (Table 2). The most common causes of olfactory loss are inflammatory in nature, occurring as a result of nasal and/or sinus disease, previous upper respiratory infection, and head trauma (7). Other potential causes of olfactory loss include toxic exposure, congenital conditions, systemic diseases, cigarette smoking, and aging itself. Despite a complete evaluation, the exact cause of olfactory loss often remains unknown (idiopathic). A number of conditions more prevalent in the elderly may contribute to chemosensory dysfunction; these include dementia, epilepsy, metabolic disorders (e.g., diabetes and hypothyroidism), and neurodegenerative disorders (e.g., Alzheimer’s and Parkinson’s) (8–11). The coexistence of multisystem disease, exposure to a broad range of toxins, and use of multiple medications also tend to be more common in the elderly. These factors taken in combination may affect chemosensory function and contribute to olfactory deficits (12). PATHOPHYSIOLOGY OF OLFACTORY LOSS WITH AGING A large body of literature indicates that the sense of smell declines with increasing age (1,3,6,13). Age-related changes to both the peripheral and central processes of olfaction are thought to be due to cumulative environmental insults with an associated reduction in regenerative capacity. Areas of the olfactory neuroepithelium, olfactory bulbs, and brain associated with olfactory processing show neuropathological changes with age. Some of these are more prominent in Alzheimer’s disease and other neurodegenerative disorders. The relationship of olfactory loss and Alzheimer’s disease is discussed in detail elsewhere in this text and will not be discussed here. Indeed, some have suggested that olfaction may be a good indicator of the integrity of the aging brain (14). The olfactory neuroepithelium is unique in its ability to replace olfactory neurons throughout the lifetime of an organism. However, loss of olfaction suggests that the system is not perfect. Animal studies have shown an age-related decline in the number of olfactory receptor neurons and atrophy of the olfactory bulb (15). In part, this decline

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Table 2 Etiology of Olfactory Dysfunction (Partial List) Nasal and/or sinus inflammatory disease Allergic rhinitis Bacterial rhinosinusitis Viral rhinosinusitis Nasal polyposis Neoplasms (benign or malignant) Post upper respiratory tract viral infection Trauma Head injury Surgical Toxic exposure Age Neurologic disease Alzheimer’s disease Parkinson’s disease Huntington’s chorea Multiple sclerosis Dementia Schizophrenia Seizures Metabolic Diabetes Hypothyroidism Liver disease Renal failure Medications Cigarette smoking Radiation therapy to the head and neck Malnutrition Congenital (Kallman’s syndrome) Idiopathic

may be due to an increased rate of olfactory receptor neuron cell death (16). Histopathologic studies of human tissue have revealed that in aging, the olfactory neuroepithelium is gradually replaced with respiratory epithelium in a patchy distribution (17). More centrally, human olfactory bulbs show atrophy with age (18). A decline in the number of mitral cells and glomeruli within the human olfactory bulb at an approximate rate of 10% per decade has also been noted during aging and may in part underlie the decline in olfactory abilities noted with age (19). Neuropathy in nondemented older adults has been described in the hippocampus, anterior olfactory nucleus, and entorhinal cortex (20). These regions are all involved in the central processing of olfactory information. Magnetic resonance imaging (MRI) has revealed age-related volume loss in areas of the brain associated with olfactory function (21,22). Functional MRI imaging studies in a cohort of elderly (mean age of 73) and young (mean age of 24) patients exposed to pulsed odorant stimuli revealed that younger subjects had a greater number of activated voxels in the regions of the frontal lobes, perisylvian, and cingulate gyri in comparison to elderly individuals (23). The association of aging and dementia must be considered when determining olfactory change, as dementia is associated with impaired olfactory function (11,24). The incidence of dementia approaches nearly 50% beyond the age of 80.

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While many earlier studies did not take this into account, more recent studies have attempted to focus on the differences in olfactory impairment between healthy aging subjects and those with dementia and other neurodegenerative disorders.

PATIENT EVALUATION History A presenting complaint of olfactory deficit in the geriatric population should be taken seriously, and not be dismissed as part of the normal aging process. A complete history should be elicited, including the degree of olfactory loss and/or the presence of a distorted perception, as well as a description of sudden versus gradual onset. Although many patients will present with a chief complaint of ageusia (loss of taste) rather than anosmia, it should be noted that the majority of taste complaints actually refer to a distortion of flavor perception, which in large part is dependent on olfaction. Patients who complain of loss of taste should be questioned specifically about the ability to distinguish between the four tastants: bitter, salty, sour, and sweet. If this perception remains intact, the problem is likely olfactory in nature. In a study of 750 patients presenting to the University of Pennsylvania Smell and Taste Center, 78.1% complained of a reduced ability to smell and 66.4% of a reduced ability to taste. After chemosensory testing was performed, however, less than 3% of patients had a taste loss, while 79.9% had an olfactory deficit (25). The history should be directed at eliciting common causes of olfactory dysfunction. Patients should be questioned about nasal and sinus disease, including allergic rhinitis, chronic sinusitis, nasal polyps, and previous nasal or sinus surgery. A history of a preceding viral upper respiratory tract infection or head trauma should be elucidated. Concomitant medical conditions and current medications should be carefully reviewed. A history of toxic exposures or chronic exposures in the home or workplace is important. Smoking and previous radiation therapy to the head and neck may also be contributing factors. Physical Examination A complete head and neck examination should be performed. This includes both anterior rhinoscopy and nasal endoscopy to evaluate for the presence of allergic rhinitis, chronic rhinosinusitis, nasal polyps, masses, or other signs of inflammation. Signs of atrophic or vasomotor rhinitis should be noted. If the history does not support a diagnosis of toxic exposure, or postviral or traumatic loss, and the physical examination does not support the presence of nasal or sinus disease, a full neurologic examination is important. As previously noted, olfactory deficits are found in a variety of neurological disorders, although they are seldom the primary complaint. Focal neurological findings may suggest the diagnosis of a central neoplastic process or stroke. Chemosensory Testing Olfactory function testing is an important step in evaluation of the geriatric patient with an olfactory complaint and usually consists of identification of specific odors, threshold detection, or both. As mentioned earlier, self-reporting alone significantly underestimates the prevalence of olfactory loss in older adults (6).

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Several clinical olfactory identification tests have been developed, which can be administered in the office. The most widely used is the University of Pennsylvania Smell Identification Test (UPSIT, commercially available as the Smell Identification TestTM, Sensonics, Inc., Haddon Heights, New Jersey, U.S.), a four-choice ‘‘scratch and sniff’’ odor identification test. Advantages of the UPSIT include the fact that it is self-administered, well validated, and based on a large body of normative data for males and females grouped according to age (26). Olfactory function can also be assessed by threshold testing, which is used to measure the detection threshold (the lowest odorant concentration where a stimulus can be perceived). The two types of detection threshold testing currently used in clinical practice include the ascending method of limits and the single staircase; in both of these, odorants are presented to the patient in increasing concentrations. In the ascending method of limits procedure, stimuli are presented in increasing concentrations from undetectable levels to those concentrations where detection is reliably made. In the single staircase method, the stimulus concentration is increased following trials on which a subject fails to detect the stimulus and decreased following trials where correct detection occurs. Commonly used stimulants are phenyl ethyl alcohol and butanol, which have little trigeminal stimulation. Patients must choose between the solution with the stimulant, and one with water only. An advantage of this type of testing is that it allows for unilateral assessment. (For a more detailed description of the procedures, see Refs. 27,28). Radiological Evaluation Radiological evaluation is not always necessary in the evaluation of olfactory loss, but can be useful when the etiology is unclear. Computed tomographic scans of the paranasal sinuses are the most useful study when a conductive olfactory loss is suspected. Coronal computed tomographic scans of the paranasal sinuses provide the clearest detail of the bony and soft tissue anatomy and can demonstrate inflammatory disease or mass lesions of the nasal cavity and sinuses. MRI is the study of choice when an intracranial lesion is suspected. Olfactory groove meningioma, frontal lobe gliomas, and other lesions of the anterior cranial fossa can produce a gradually progressive olfactory loss, although it should be noted that such lesions rarely present with olfactory loss as their only symptom. Other Diagnostic Tests Routine laboratory tests are rarely helpful in the evaluation of the patient with olfactory loss. However, when clinical suspicion warrants, laboratory tests may help to confirm diagnoses such as hypothyroidism, diabetes, or renal or liver failure. It has been shown that some vitamin and mineral deficiencies such as B12, zinc, and copper can be associated with olfactory or taste dysfunction, but these causes are so rare that evaluation for such deficiencies is rarely indicated unless patients demonstrate other clinical signs of severe malnutrition. TREATMENT AND COUNSELING In general, treatment of anosmia or hyposmia is extremely limited. Only olfactory loss due to nasal or sinus inflammatory disease is readily amenable to treatment, which is usually directed at controlling allergic rhinitis, or treating rhinosinusitis.

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Nasal polyps are usually addressed initially with trials of topical and/or oral steroids. Surgery may be indicated in cases of anatomical obstruction, rhinosinusitis unresponsive to medical management, and severe nasal polyposis. A short trial of oral steroids can sometimes be helpful in determining the presence of reversible olfactory dysfunction secondary to inflammatory disease. In the rare instance that olfactory loss is thought to be due to an underlying medical condition, the latter should be corrected. Unfortunately, no effective treatment for postviral olfactory loss or olfactory loss due to head trauma is available. It is believed by many physicians that postviral olfactory loss that persists through the first year is permanent. However, in a long-term study following patients with postviral loss, 62–90% of patients showed some improvement in olfactory function at five years, depending on whether subjective or objective measures were assessed (29). Reduced regenerative ability of the aging olfactory epithelium to recover function after damage by a viral infection may explain the higher incidence of postviral olfactory deficits in the elderly population. The prognosis for patients with olfactory loss secondary to head trauma is poor and most post-traumatic deficits are permanent. Regardless of etiology, appropriate counseling with patients and their family members is important in the management of olfactory deficits. This may be even more crucial in the elderly patient who may live alone and suffer from impairment of other sensory functions as well. Patients must be specifically counseled with regards to hazards such as smoke or leaking gas; installation and maintenance of working smoke detectors is therefore essential. Gas appliances should be equipped with natural gas detectors or changed in favor of electric ones whenever possible. These devices should be regularly checked and serviced by family members or facility personnel. Unfortunately, a disproportionate number of elderly dies from accidental gas poisoning (30). Patients are also at risk for food poisoning and must be counseled about spoiled items. They must adhere to a vigilant leftover policy and food should be regularly verified by a family member or friend. Nutrition is another area of concern for older persons with olfactory loss. Since olfaction plays a large role in the perception of flavor, patients with diminished olfaction may have decreased enjoyment of food. Whether this influences overall food intake in the elderly population has yet to be fully determined, but in a population that may also be susceptible to changes in food intake regulatory mechanisms, the risk for nutritional deficits may be increased (31). As in other chronic medical conditions, olfactory impairment may have a significant impact on quality of life. Results of a recent retrospective questionnaire sent to individuals with documented olfactory loss indicated a lower quality of life and higher level of disability in individuals with continued olfactory loss compared those with resolution of previous olfactory compromise (32). It is estimated that approximately 14 million older adults in the United States have impairment of olfactory function. Although loss of olfactory function may occur as part of the normal aging process, it has been shown that older patients are largely unaware of their olfactory impairment. The impact of olfactory loss on the elderly patient may be especially great, when coexistent with diminished function in the other senses, or other systemic or physiologic changes. Perceived olfactory loss by an older individual should always be fully evaluated and treated when possible. Special attention should be devoted to appropriate counseling regarding the inability to detect hazards such as smoke, gas leaks, and spoiled foods. Reassurance and acknowledgement of the severity of impairment and impact on quality of life are also important components of management.

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REFERENCES 1. Cain WS, Stevens JC. Uniformity of olfactory loss in aging. Ann N Y Acad Sci 1989; 561:29–38. 2. Cowart BJ. Relationships between taste and smell across the adult life span. Ann N Y Acad Sci 1989; 561:39–55. 3. Doty RL, Shaman P, Applebaum SL, et al. Smell identification ability: changes with age. Science 1984; 226:1441–1443. 4. Hoffman HJ, Ishii EK, MacTurk RH. Age-related changes in the prevalence of smell/ taste problems among the United States adult population. Results of the 1994 disability supplement to the National Health Interview Survey (NHIS). Ann N Y Acad Sci 1998; 855:716–722. 5. Nordin S, Monsch A, Murphy C. Unawareness of smell loss in normal aging and Alzheimer’s disease: discrepancy between self-reported and diagnosed smell sensitivity. J Gerontol B Psychol Sci Soc Sci 1985; 50:187–192. 6. Murphy C, Schubert CR, Cruickshanks KJ, et al. Prevalence of olfactory impairment in older adults. JAMA 2002; 288:2307–2312. 7. Cowart BJ, Young IM, Feldman RS, et al. Clinical disorders of smell and taste. [review] [117 refs]. Occupational Med 1997; 12:465–483. 8. Royall DR, Chiodo LK, Polk MS, et al. Severe dysosmia is specifically associated with Alzheimer-like memory deficits in nondemented elderly retirees. Neuroepidemiology 2002; 21:68–73. 9. Hawkes CH, Shephard BC, Daniel SE. Is Parkinson’s disease a primary olfactory disorder? [review] [60 refs]. Q J Med 1999; 92:473–480. 10. Hawkes C. Olfaction in neurodegenerative disorder [review] [68 refs]. Movement Disord 2003; 18:364–372. 11. Murphy C. Loss of olfactory function in dementing disease [review] [59 refs]. Physiol Behav 1999; 66:177–182. 12. Doty RL. Olfaction. Annu Rev Psychol 2001; 52:423–452. 13. Wysocki CJ, Gilbert AN. National Geographic Smell Survey. Effects of age are heterogeneous. Ann N Y Acad Sci 1989; 561:12–28. 14. Dulay MF. Olfactory acuity and cognitive function converge in older adulthood: support for the common cause hypothesis [article]. Psychol Aging 2002; 17:392–404. 15. Hinds JW, McNelly NA. Aging in the rat olfactory system: correlation of changes in the olfactory epithelium and olfactory bulb. J Comparative Neurol 1981; 203:441–453. 16. Conley DB, Robinson AM, Shinners MJ, et al. Age-related olfactory dysfunction: cellular and molecular characterization in the rat. Am J Rhinol 2003; 17:169–175. 17. Paik SI, Lehman MN, Seiden AM, et al. Human olfactory biopsy. The influence of age and receptor distribution. Arch Otolaryngol Head Neck Surg 1992; 118:731–738. 18. Smith CG. Incidence of atrophy of the olfactory nerves in man. Arch Otolaryngol Head Neck Surg 1941; 34:533–539. 19. Meisami E, Mikhail L, Baim D, et al. Human olfactory bulb: aging of glomeruli and mitral cells and a search for the accessory olfactory bulb. Ann N Y Acad Sci 1998; 855:708–715. 20. Price JL, Davis PB, Morris JC, et al. The distribution of tangles, plaques and related immunohistochemical markers in healthy aging and Alzheimer’s disease. Neurobiol Aging 1991; 12:295–312. 21. Jernigan TL, Archibald SL, Fennema-Notestine C, et al. Effects of age on tissues and regions of the cerebrum and cerebellum. Neurobiol Aging 2001; 22:581–594. 22. Cerf-Ducastel B, Murphy C. FMRI brain activation in response to odors is reduced in primary olfactory areas of elderly subjects. Brain Res 2003; 986:39–53. 23. Yousem DM, Maldjian JA, Hummel T, et al. The effect of age on odor-stimulated functional MR imaging. Am J Neuroradiol 1999; 20:600–608.

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24. Morgan CD, Nordin S, Murphy C. Odor identification as an early marker for Alzheimer’s disease: impact of lexical functioning and detection sensitivity. J Clin Exp Neuropsychol 1995; 17:793–803. 25. Deems DA, Doty RL, Settle RG, et al. Smell and taste disorders, a study of 750 patients from the University of Pennsylvania Smell and Taste Center. Arch Otolaryngol Head Neck Surg 1991; 117:519–528. 26. Doty RL, Shaman P, Dann M. Development of the University of Pennsylvania smell Identification Test: a standardized microencapsulated test of olfactory function. Physiol Behav 1984; 32:489–502. 27. Doty RL. Practical approaches to clinical olfactory testing. In: Seiden AM, ed. Taste and Smell Disorders. New York: Thieme, 1997:38–51. 28. Seiden AM, Duncan HJ, Smith DV. Office management of taste and smell disorders. Otolaryngol Clin North Am 1992; 25:817–835. 29. Duncan HJ, Seiden AM. Long-term follow-up of olfactory loss secondary to head trauma and upper respiratory tract infection. Arch Otolaryngol Head Neck Surg 1995; 121:1183–1187. 30. Chalke H, Dewhurst J, Ward C. Loss of sense of smell in old people: a possible contributory factor in accidental poisoning from town gas. Publ Health 1958; 72:223–230. 31. Rolls BJ. Do chemosensory changes influence food intake in the elderly? [review] [35 refs]. Physiol Behav 1999; 66:193–197. 32. Miwa T, Furukawa M, Tsukatani T, et al. Impact of olfactory impairment on quality of life and disability. Arch Otolaryngol Head Neck Surg 2001; 127:497–503.

16 Olfactory Dysfunction in Neurodegenerative Diseases Richard L. Doty Smell and Taste Center, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, U.S.A.

INTRODUCTION While it is widely known that the sense of smell declines with age (Fig. 1), it is less well known that this sense is altered in a number of neurodegenerative diseases whose phenotypes often become expressed in mid- to late-life, including Alzheimer’s disease (AD), Huntington’s disease (HD), idiopathic Parkinson’s disease (PD), multiple sclerosis (MS), and the parkinsonism-dementia complex of Guam (PDC). Smell loss is also found in disorders associated with cerebellar degeneration (e.g., Friedreich’s ataxia), and is a hallmark of schizophrenia (SZ), a disease commonly viewed as neurodevelopmental (for review, see Ref. 1). However, considerable variation in the prevalence and magnitude of olfactory dysfunction is present among such disorders. For example, AD, PD, and PDC are accompanied by marked alterations in the ability to smell, whereas HD, multi-infarct dementia, and SZ are accompanied by more moderate alterations. Progressive supranuclear palsy (PSP) and 1-methyl-4phenyl-l,2,3,6-tetrahydropyridine–induced parkinsonism (MPTP-P) are associated with only minor, if any, changes in the ability to smell, in spite of the fact that they share major clinical features with PD. It is not known to what degree the age-related decrements shown in Figure 1 reflect the earliest stages of an age-related neurodegenerative disease. Presumably a significant number of older persons experiencing smell dysfunction are, in fact, within the so-called preclinical stages of one or more of these diseases. Thus, when a nasal examination proves negative and smell loss is present in an older patient, it behooves the otorhinolarngologist to inquire about possible changes in motor function and cognition. If anomalies are noted by the patient or a close relative, or become apparent during the medical examination, referral to a neurologist or a memory disorders specialist may be in order. In the case of AD, there is both human and animal evidence that pharmacological interventions (e.g., with antioxidants, such as melatonin and vitamins C and E, as well as cholinergic agonists), if given early in the disease process, may mitigate to some degree the progression of elements of the disease process (e.g., Ref. 2). 181

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Figure 1 UPSIT scores as a function of age in a large heterogeneous group of subjects. Numbers by data points indicate sample sizes. Abbreviation: UPSIT, University of Pennsylvania Smell Identification Test. Source: From Ref. 3.

This chapter reviews the major neurodegenerative disorders for which olfactory dysfunction is present and provides details of the nature and magnitude of the dysfunction. The reader is referred elsewhere for a review of the possible neuropathological bases of the olfactory anomalies observed in such disorders (4).

NEURODEGENERATIVE DISEASES WITH MARKED OLFACTORY DYSFUNCTION Alzheimer’s Disease AD is a pathological diagnosis made at autopsy. Nonetheless, the term ‘‘Alzheimer’s disease’’ is commonly used synonymously with the term ‘‘probable Alzheimer’s disease,’’ which is diagnosed during life using a well-defined set of clinical inclusion and exclusion criteria (e.g., among others, an idiopathic slowly developing memory loss). Although olfactory dysfunction is perhaps the best predictor of conversion from mild cognitive decline to a diagnosis of probable AD, smell loss has not yet achieved orthodoxy in this regard. Much is known about the olfactory phenotype of AD, although only recently have we begun to understand the physiological causes of the olfactory losses. Key features of the olfactory dysfunction are as follows: First, the loss, which is usually not total, typically presents bilaterally (i.e., in both nasal chambers). Second, it is

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present in 85–90% of early-stage patients. Third, its magnitude is generally greater in men than in women. Fourth, it is robust (Fig. 2) and detectable by a wide range of olfactory tests, including tests of odor identification, detection, discrimination, and memory. In a meta-analysis of 11 olfactory/AD studies, for example, effect sizes ranging from 0.98 to 12.15 (median ¼ 2.17) were found for tests of odor identification, detection, and memory (5). Fifth, many AD patients are unaware of their olfactory deficit until formal testing. In one study, for example, only 6% (2/34) of early-stage AD patients responded affirmatively to the question, posed before olfactory testing, ‘‘Do you suffer from smell and/or taste problems?’’ despite the fact that over 90% exhibited lower scores on the 40-item University of Pennsylvania Smell Identification Test (UPSIT) than age-matched controls (6). Sixth, the olfactory loss of AD appears, on average, to be equivalent to that observed in PD and PDC, suggesting the possibility of a shared pathological substrate. Seventh, progression of dysfunction over time may occur in AD, conceivably reflecting the progression of pathology. Eighth, the smell loss observed in AD is often not present in disorders commonly misdiagnosed as AD, such as major affective disorder. Even a simple three-item odor

Figure 2 (A) UPSIT scores for patients with AD and for age-, gender-, and race-matched controls. (B) Detection threshold values for phenylethyl alcohol for AD patients and controls. Each dot signifies an individual subject’s data point. Although some overlap appears between the AD and control subject data when plotted in this manner, very few of the AD subjects performed better than their matched controls. Abbreviations: UPSIT, University of Pennsylvania Smell Identification Test; AD, Alzheimer’s disease. Source: From Ref. 6.

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identification test more accurately makes the distinction between AD and depression than the widely used 30-item Mini-Mental State Examination (MMSE) (7). Ninth, genetic factors are involved in the smell loss of at least some cases of AD and smell loss seems to be one of the best predictors of future AD in at-risk populations. Thus, olfactory dysfunction (a) is present in some inherited forms of AD, (b) correlates with a family history of dementia, and (c) is more likely to occur in individuals with the apolipoprotein E (APOE) genotype. In a now classic study, Graves et al. (8) administered the 12-item Brief Smell Identification TestTM (B-SIT) to 1604 nondemented community-dwelling senior citizens 65 years of age or older. Over a subsequent two-year time period, the B-SIT scores were found to be a better predictor of cognitive decline than scores on a global cognitive test. Persons who were anosmic and possessed at least one APOE-4 allele had 4.9 times the risk of having cognitive decline than normosmic persons not possessing this allele. This is in contrast to the 1.23 times greater risk for cognitive decline in normosmic individuals possessing at least one such APOE allele. When the data were stratified by sex, women who were anosmic and possessed at least one APOE-4 allele had an odds ratio of 9.71, compared to an odds ratio of 1.90 for women who were normosmic and possessed at least one allele. The corresponding odds ratios for men were 3.18 and 0.67, respectively. Down Syndrome Down syndrome (DS), a trisomy 21 disorder, accounts for ~17% of the mentally challenged population. A number of studies have demonstrated olfactory deficits in DS adults (for review, see Ref. 2). Given that the average smell loss observed in DS is similar to that observed in AD (i.e., UPSIT scores ~20), and DS patients who live into early adulthood inevitably develop the clinical and neuropathological features of AD, the question arises as to whether the smell loss is secondary to the AD-like neuropathology. One means of addressing this question is to determine at what age the olfactory loss expresses itself in DS. McKeown et al. (9) demonstrated olfactory dysfunction in 20 young DS subjects [mean age (SD) ¼ 13.89 years (1.98)] equivalent to that observed in adult DS subjects. However, non-DS retarded children of the same IQ also exhibited such dysfunction. Thus, the olfactory loss appears to somehow be associated with retardation, rather than the development of AD-like amyloid pathology per se, since amyloid deposits do not emerge within the entorhinal cortex of DS subjects until ~19 years of age (10). Parkinson’s Disease PD, since its first description by James Parkinson in 1817, has generally been considered to be a purely motor disease. It is now known, however, that some sensory changes accompany this disorder, including subtle alterations in vision and hearing and marked changes in olfaction. Like AD, much is known about the olfactory phenotype of PD. First, it occurs in ~90% of all cases, a frequency higher than that of tremor, one of the cardinal signs of the disorder (Fig. 3). In one study, the sensitivity and specificity of the UPSIT in differentiating between clinically diagnosed PD male patients and normal controls under the age of 61 was 0.91 and 0.88, respectively (11). Second, as in the case of AD, female PD patients generally have less dysfunction than male PD patients. Third, also as in AD, the loss is usually less than total and in some cases reflects difficulty in identifying, rather than detecting, the stimulus. Thus, in one report only 13% of 38 patients who received an odor detection threshold test

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Figure 3 (A) UPSIT scores of PD patients and matched normal controls. (B) Phenyl ethyl alcohol odor detection threshold values of PD patients and matched normal controls. Abbreviations: UPSIT, University of Pennsylvania Smell Identification Test; PD, Parkinson’s disease. Source: From Ref. 12.

were unable to detect the highest odorant concentration presented, and only 38.3% of 81 patients had UPSIT scores suggestive of anosmia (12). All but one of 41 PD patients asked if an odor was present on each of 40 UPSIT items answered affirmatively to 35 or more of the items, despite the fact that most were unable to identify the majority of the odors or felt that the perceived sensation did not correspond to the response alternatives. Fourth, the loss is generally bilateral, although there can be slight individual differences in the degree to which the left and right sides of the nose are involved. No association exists, however, between the side of relatively greater involvement and the side of hemiparkinsonism, as might be expected if asymmetrical damage to striatal dopamine systems were involved in the olfactory problem. Fifth, the smell loss is indistinguishable from that of AD and PDC, with UPSIT scores averaging around 20. Sixth, the smell loss is unrelated to the magnitude of the motor symptoms, although subtle variations among the so-called benign versus malignant forms may be present. Seventh, the smell loss is unrelated to performance on numerous neuropsychological measures, such as the Randt memory test, reaction time, a finger-tapping test, and selected verbal and performance subsets of the Wechsler Adult Intelligence Scale—revised (13). Eighth, the smell loss appears in both familial and sporadic forms of parkinsonism and may be a sign of the preclinical state of the disease. Ninth, unlike AD, there is no clear longitudinal progression in olfactory dysfunction as occurs in other elements of the disease process. Tenth, anti-PD medications (e.g., L-dopa, dopamine agonists, anticholinergic compounds) have no influence on the smell deficit, which occurs as severely in nonmedicated or never-medicated patients as in medicated ones. Eleventh, also like AD, many PD patients are unaware of their olfactory deficit until formal testing (12). Twelfth, olfactory testing is useful in the differential diagnosis of idiopathic PD from a number of other neurodegenerative diseases with motor symptoms, including disorders often misdiagnosed as PD (e.g., PSP, MPTP-induced PD, and essential tremor). Finally, as in the case with AD, some asymptomatic first-degree

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relatives of patients with either familial or sporadic forms of PD appear to exhibit olfactory dysfunction. Recently, Berendse (14) administered tests of odor detection, identification, and discrimination to 250 relatives of PD patients (~84% children, ~16% siblings, one parent). In 25 hyposmic and 23 normosmic individuals sampled from this group, nigrostriatal dopaminergic function was assessed using single photon emission computer tomography with [125I]b-CIT as a dopamine transporter ligand. An abnormal reduction in striatal dopamine transporter binding was present in four of the 25 (16%) hyposmic relatives, two of whom subsequently developed clinical parkinsonism, and in none of the 23 normosmic relatives. These authors noted (p. 39), ‘‘The observation in the present study that significant reductions in dopamine transporter binding were found only in hyposmic relatives of PD patients suggests that olfactory dysfunction may precede clinical motor signs in PD.’’ Such findings reiterate the point that olfactory testing, in conjunction with other measures, is likely useful in the early detection of PD. Parkinsonism-Dementia Complex of Guam Between 1957 and 1965, amyotrophic lateral sclerosis and parkinsonism-dementia accounted for at least 15% of adult deaths among the Chamorro populations of Guam and Rota (15). Although poorly understood, genetic susceptibility may be involved in producing this disorder. Even though no differences in APOE-4 frequencies have been found, persons with PDC have a lower frequency of APOE-2 than do controls (16). Guamanian Chamorros with PDC have olfactory deficits similar to those observed in AD and PD. In one study, 24 PDC patients were administered the UPSIT (17). All were ambulatory and living with their families. At the time of testing, all evidenced some degree of rigidity and bradykinesia. Their scores did not differ significantly from those of 24 AD and 24 PD North American patients of similar age, gender, and smoking history. However, their scores were markedly lower than normals of the same age and gender. As in earlier studies, each subject was asked, prior to olfactory testing, whether or not he or she suffered from any smell or taste problems. Three of the PDC patients reported such problems (13%), as compared to two of the AD (8%) and three of the PD (13%) patients, indicating that the level of awareness of the problem is similar, if not identical, in these three disorders. In a more recent study, an abbreviated version of the UPSIT was administered to nine Chamorros with symptoms of amyotrophic lateral sclerosis (ALS) alone, nine with symptoms of pure parkinsonism, 11 patients with pure dementia, and 31 patients with PDC, as well as to neurologically normal Chamorro Guamanians and 25 North American controls (18). The UPSIT scores were markedly depressed in the four disease groups relative to the controls, and did not differentiate among the four syndromes. Some of the control subjects had lower scores than North American counterparts, suggesting the possible presence of a subclinical neurogenerative disease process in nonsymptomatic individuals. Huntington’s Disease HD, a progressive disorder of dysfunctional movement, cognitive deterioration, and altered behavior with autosomal dominant transmission, is often phenotypically expressed relatively late in life. As functional capacity worsens, chorea generally lessens and dystonia intensifies. Among its primary motor symptoms are hyperkinesias that take, initially, the form of chorea, being characterized by fleeting movements

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which, in some cases, appear semi-purposively within the context of overall heightened activity and motor restlessness. A number of studies have shown that HD patients exhibit deficits in odor identification, detection, discrimination, and memory, and that the problem is likely manifested by the time the classical phenotypic elements of the disorder appear. However, when the olfactory function occurs during the progression of the disease process is not presently known. In one study, UPSIT and phenyl ethyl alcohol (PEA) detection threshold scores were obtained from 25 probands with HD, 12 at-risk offspring, and 37 unrelated controls. Decreased olfaction was noted only in the HD group, with a mean UPSIT score of 24.8 (SD ¼ 8.7) and a PEA threshold score of
4.4 log vol/vol (SD ¼ 1.4) (19). More recently, these findings were extended by testing 20 HD patients who had the disease for a mean of eight years (range: 4–14 years), 20 normal subjects with the genetic mutation that causes HD, and 20 mutation-negative adults. Again, only the patients with clinical signs of HD exhibited depressed olfaction (mean UPSIT score ¼ 27.4, SD ¼ 6.5) (20).

NEURODEGENERATIVE DISEASES WITH LESS MARKED OR MINIMAL OLFACTORY DYSFUNCTION 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine–Induced Parkinsonism MPTP-P represents one syndrome with a PD-like phenotype that is not accompanied by significant alterations in olfactory function. Unlike most of the other disorders described in this chapter, the factor responsible for the neurological damage of patients with MPTP-P is well established. In this case, intravenous injection of MPTP, whose toxic metabolite 1-methyl-4-phenylpyridinium (MPPþ) crosses the blood–brain barrier and damages brain regions critical for the coordination of motor movement, produces the parkinsonian symptoms (21). In the sole study addressing olfactory function in MPTP-P, UPSIT scores and detection threshold values for the odorant phenyl ethyl alcohol in six such patients did not differ significantly from those of 10 normal subjects of equivalent age (22). Thirteen rare young PD patients exhibited significantly lower test scores than both the MPTP-P and control subjects. Despite their major motor deficits, the MPTP-P patients evidenced no major decrements in cognitive function, could sniff adequately, and could respond verbally to the examiner’s questions without difficulty. These data suggest that the functional integrity of the olfactory system of MPTP-P patients is greater than that of PD patients and demonstrate that olfactory dysfunction need not always be a concomitant element of parkinsonian syndromes. Progressive Supranuclear Palsy PSP (also termed the Steele, Richardson, and Olszewski syndrome) accounts for ~4% of patients with parkinsonian symptoms. Tremor is rarely present, although rigidity and bradykinesia frequently appear early in disease progression. The hallmark feature is vertical-gaze paresis (especially down-gaze paresis), which is of supranuclear origin and can be overcome by the oculocephalic maneuver. Commonly misdiagnosed as PD, PSP shares many motor features with PD. The parkinsonian features are, however, less responsive to anti-PD medications, and PSP typically has comparatively more frontal lobe dysfunction, more neuronal degeneration within the basal ganglia and upper brain stem, and less involvement of mesolimbic and mesocortical dopamine systems than PD (23).

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Most patients with PSP have a relatively normal sense of smell, although slight to moderate losses are present in some individuals. In one study, the UPSIT and a phenyl ethyl alcohol odor detection threshold test were administered to 22 patients with PSP (24). The test scores of these individuals were compared to those from 22 PD patients and 22 neurologically normal age-, gender-, and race-matched controls. The performance was markedly superior to that of the PD patients [respective UPSIT means (SD): 31.59 (7.18) and 18.82 (6.94)], with approximately half scoring within the normal range. However, on average, the PSP patients did exhibit moderate deficits relative to the controls, whose mean (SD) UPSIT score was 35.60 (4.06). Multiple System Atrophy Wenning et al. (25) administered the UPSIT to 29 patients with multiple system atrophy (MSA), 15 PSP patients, 118 patients with idiopathic PD, seven patients with corticobasal degeneration (CBD), and 123 healthy controls. Relative to controls, the MSA patients had mild impairment. Normal test scores were noted for the PSP and CBD patients. The authors noted that ‘‘preserved or mildly impaired olfactory function in a parkinsonian patient is more likely to be related to atypical parkinsonism such as MSA, PSP, or CBD, whereas markedly reduced olfaction is more suggestive of idiopathic Parkinson’s disease (IPD).’’ An UPSIT score of 25 was associated with a sensitivity of 77% and a specificity of 85% in differentiating PD from atypical parkinsonism. Multiple Sclerosis Until the mid-1980s, it was generally assumed that olfactory function was not altered at all in MS, since the primary olfactory neurons are unmyelinated. Indeed, the sole psychophysical study available at that time suggested no deficits were present (26). In 1984, we reported that 23% of 31 MS patients evaluated evidenced some degree of olfactory dysfunction on the UPSIT (27). A decade later, we presented case studies in which olfactory dysfunction was the presenting symptom of MS (28), and, more recently, demonstrated a strong inverse correlation (r ¼
0.94) between UPSIT scores and the number of MS-related plaques within the subfrontal and subtemporal lobes of patients (Fig. 4) (29,30). No such relationship was present between UPSIT scores and plaques in other brain regions. Subsequently, it has been shown that, in individual cases, UPSIT scores wax and wane in concert with the fluctuations in the number of plaques within olfaction-related brain regions (31). ALS As with the case of PD, ALS has been traditionally considered a motor neuron disease (MND). However, today we know that this is an oversimplification of this disorder. In 1991, Elian (32) reported that patients with MND exhibit decreased UPSIT scores bilaterally. Specifically, UPSIT scores from nine male and six female MND patients of varying severity were compared to those of age- and sex-matched controls, and proved to be significantly depressed. However, the magnitude of the deficit was more similar to that observed in schizophrenia than that observed in AD or PD, with the UPSIT scores falling around 30. In a subsequent study, Sajjadian et al. (33) administered the UPSIT bilaterally to 17 female and 20 male ALS patients, and unilaterally to seven male and seven

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Figure 4 Relationship, in patients with MS, between number of plaques in subtemporal and subfrontal lobes and scores on the UPSIT. Abbreviations: UPSIT, University of Pennsylvania Smell Identification Test; MS, multiple sclerosis. Source: From Ref. 29.

female ALS patients. Age-, gender-, smoking-habit-, and race-matched controls were also evaluated. While the UPSIT scores of the ALS patients were significantly lower than those of the controls, the degree of dysfunction was similar to that seen by Elian. Thus, only 11% of the ALS patients had UPSIT scores indicative of total or near total anosmia. Despite the fact that nearly half of the ALS patients had UPSIT scores that fell within the normal range, 75.7% of the patients scored below their individually matched controls. Although no sex differences or laterality in the ALS-related test scores were observed, an age-related decrement was present and, interestingly, significant correlations were found between UPSIT scores and neurophysiological measures of peripheral nerve conductance. If the latter observation proves to be true, it is conceivable that a common pathophysiological process influences the motor neuron responses and segments of the afferent olfactory pathway. More recently, Hawkes et al. (34) administered the UPSIT to 58 patients with MND and 135 controls. Additionally, olfactory event-related potentials (OERPs) in response to hydrogen sulfide (H2S) were recorded in 15 patients, and the olfactory bulbs of eight MND cadavers were histologically examined. Although UPSIT scores were slightly worse in the MND patients, only the bulbar patients exhibited significant decrements. OERPs were normal in nine patients and delayed in one. OERPs from the remaining five subjects could not be recorded. Histological analysis of olfactory bulb tissue revealed excessive lipofuscin deposition in eight of eight cases examined.

Epilepsy The earliest threshold studies of patients with epilepsy reported heightened, not lessened, overall bilateral sensitivity, particularly prior to an ictal event (e.g. Ref. 35).

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This has not been observed in more recent studies, where detection thresholds for n-butanol and phenyl ethyl alcohol have been found normal (36). Suprathreshold deficits, however, have been reported, with several studies suggesting right-side foci are more disrupting. Thus, epileptic patients with right-side foci, but not left-side foci, have been found to exhibit decreased performance on an odor matching task, an odor memory test for nameable odors, and the UPSIT. Bilateral deficits have been reported for odor discrimination, short- and long-term odor memory, and odor naming (36). Prolonged odor event-related potential latencies have been reported in patients with both right- and left-side foci when the stimulation was made on the focal side (37).

CONCLUSIONS It is apparent from the studies reviewed in this chapter that olfactory loss is a common element of many neurodegenerative diseases. Moreover, it is clear that the losses are (a) highly variable across diseases, (b) appear, in some cases, very early in the disease process, and (c) may or may not relate to disease stage, depending upon the disorder. As reviewed elsewhere (1), well over a hundred studies have now been published in the peer-reviewed literature demonstrating adverse influences of these diseases on the ability to smell. Although the present study provides considerable information with respect to the olfactory dysfunction seen in a number of neurodegenerative diseases, it should be emphasized that this review is not comprehensive. At least some smell dysfunction has been demonstrated in a number of neurodegenerative disorders that are not discussed in detail, including Pick’s disease, multi-infarct dementia, restless leg syndrome, and essential tremor (2). Although smell dysfunction is a hallmark of SZ, SZ is generally considered a neurodevelopmental, not a neurodegenerative, disease and for this reason was not reviewed in detail in this chapter. Nonetheless, UPSIT scores correlate inversely with the time since diagnosis, suggesting that there may be a progressive, possibly degenerative, element within brain regions associated with olfactory processing in this disease (38). While it is quite possible that the lack of olfactory threshold deficits in some diseases is a real phenomenon, caution is warranted in interpreting a number of studies of olfactory threshold. Thus, thresholds based upon a single ascending series of stimuli or using non-forced-choice methodology can be extremely unreliable. Hence, such measures may not have the power to detect differences when small samples are employed. A case in point is a study that reported significant UPSIT, but not pyridine threshold, deficits in AD patients relative to controls (39). Based on this observation, the authors concluded that the olfactory deficit of AD is associated with a ‘‘central’’ rather than a ‘‘peripheral’’ anomaly. While, ultimately, their overall conclusion may be correct (i.e., that the cause of olfactory deficits in AD reflects damage in structures more central than the olfactory neuroepithelium), the basis upon which they arrive at this conclusion is suspect for three reasons. First, the vast majority of studies find threshold deficits in AD patients, and even their own data point to this conclusion. Thus, three of their subjects (30%) were either anosmic or hyposmic by their own criteria and, in fact, the average threshold value for the AD patients was higher (i.e., indicative of less sensitivity) than that of the controls, even though statistical significance at the 0.05 level was not achieved. Second, compared to other studies on this topic (see Ref. 1), their sample size was very small, compromising

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statistical power. In general, single ascending series detection threshold tests such as administered by them have test–retest reliability values on the order of 0.40, compared to those of the UPSIT, which are uniformly above 0.90. Third, the general assumption that threshold tests reflect peripheral damage and identification tests reflect central damage is questionable. Thus, central lesions have been associated with threshold deficits in some studies (e.g., Refs. 40,41) and peripheral (i.e., neuroepithelial) lesions are known to influence both detection threshold and odor identification tests (42).

ACKNOWLEDGMENTS This work was supported, in part, by Grants RO1 DC 04278, RO1 DC 02974, RO1 AG 08148, and RO1 AG 27496 from the National Institutes of Health, Bethesda, Maryland, U.S.A. Disclosure: Dr. Doty is a major shareholder in Sensonics, Inc., the manufacturer and distributor of the UPSIT and other chemosensory test. REFERENCES 1. Doty RL. Odor perception in neurodegenerative diseases. In: Doty RL, ed. Handbook of Olfaction and Gustation. New York: Marcel Dekker, 2003:479–502. 2. Sung S, Yao Y, Uryu K, et al. Early vitamin E supplementation in young but not aged mice reduces A beta levels and amyloid deposition in a transgenic model of Alzheimer’s disease. FASEB J 2004; 18:323–325. 3. Doty RL, Shaman P, Applebaum SL, Giberson R, Siksorski L, Rosenberg, L. Smell identification ability: changes with age. Science 1984; 226:1441–1443. 4. Smutzer GS, Doty RL, Arnold SE, Trojanowski JQ. Olfactory system neuropathology in Alzheimer’s disease, Parkinson’s disease, and schizophrenia. In: Doty RL, ed. Handbook of Olfaction and Gustation. New York: Marcel Dekker, 2003:503–523. 5. Mesholam RI, Moberg PJ, Mahr RN, Doty RL. Olfaction in neurodegenerative disease: a meta-analysis of olfactory functioning in Alzheimer’s and Parkinson’s diseases. Arch Neurol 1998; 55:84–90. 6. Doty RL, Reyes PF, Gregor T. Presence of both odor identification and detection deficits in Alzheimer’s disease. Brain Res Bull 1987; 18:597–600. 7. McCaffrey RJ, Duff K, Solomon GS. Olfactory dysfunction discriminates probable Alzheimer’s dementia from major depression: a cross-validation and extension. J Neuropsychiatr Clin Neurosci 2000; 12:29–33. 8. Graves AB, Bowen JD, Rajaram L, et al. Impaired olfaction as a marker for cognitive decline: interaction with apolipoprotein E epsilon4 status. Neurology 1999; 53:1480–1487. 9. McKeown DA, Doty RL, Perl DP, Frye RE, Simms I, Mester. Olfactory function in young adolescents with Down’s syndrome. J Neurol Neurosurg Psychiatry 1996; 61: 412–414. 10. Hof PR, Bouras C, Perl DP, Sparks L, Mehta N, Morrison JH. Age-related distribution of neuropathologic changes in the cerebral cortex of patients with Down’s syndrome. Arch Neurol 1995; 52:379–391. 11. Doty RL, Bromley SM, Stern MB. Olfactory testing as an aid in the diagnosis of Parkinson’s disease: development of optimal discrimination criteria. Neurodegeneration 1995; 4:93–97. 12. Doty RL, Deems DA, Stellar S. Olfactory dysfunction in parkinsonism: a general deficit unrelated to neurologic signs, disease stage, or disease duration. Neurology 1988; 38:1237–1244.

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13. Doty RL, Riklan M, Deems DA, Reynolds C, Stellar S. The olfactory and cognitive deficits of Parkinson’s disease: evidence for independence. Ann Neurol 1989; 25:166–171. 14. Berendse HWB. Subclinical dopaminergic dysfunction in asymptomatic Parkinson’s disease patients’ relatives with a decreased sense of smell. Ann Neurol 2001; 50:34–41. 15. Reed DM, Brody JA. Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam 1945–1972. I. Descriptive Epidemiology. Am J Epidemiol 1975; 101:287–301. 16. Buee L, Perez-Tur J, Leveugle B, et al. Apolipoprotein E in Guamanian amyotrophic lateral sclerosis/parkinsonism-dementia complex: genotype analysis and relationships to neuropathological changes. Acta Neuropathol 1996; 91:247–253. 17. Doty RL, Perl DP, Steele JC, et al. Odor identification deficit of the parkinsonismdementia complex of Guam: equivalence to that of Alzheimer’s and idiopathic Parkinson’s disease. Neurology 1991; 41:77–80. 18. Ahlskog JE, Waring SC, Petersen RC, et al. Olfactory dysfunction in Guamanian ALS, parkinsonism, and dementia. Neurology 1998; 51:1672–1677. 19. Moberg PJ, Doty RL. Olfactory function in Huntington’s disease patients and at-risk offspring. Int J Neurosci 1997; 89:133–139. 20. Bylsma FW, Moberg PJ, Doty RL, Brandt J. Odor identification in Huntington’s disease patients and asymptomatic gene carriers. J Neuropsychiatr Clin Neurosci 1997; 9: 598–600. 21. Langston JW, Ballard PA, Tetrude JW, Irwin I. Chronic parkinsonism in humans due to a product of meperidine-analog synthesis. Science 1983; 219:979–980. 22. Doty RL, Singh A, Tetrude J, Langston JW. Lack of olfactory dysfunction in MPTPinduced parkinsonism. Ann Neurol 1992; 32:97–100. 23. Jankovic J. Parkinsonism-plus syndromes. Movement Dis 1989; 4:S95–S119. 24. Doty RL, Golbe LI, McKeown DA, Stern MB, Lehrach CM, Crawford D. Olfactory testing differentiates between progressive supranuclear palsy and idiopathic Parkinson’s disease. Neurology 1993; 43:962–965. 25. Wenning GK, Shephard B, Hawkes C, Petruckevitch A, Lees A, Quinn N. Olfactory function in atypical parkinsonian syndromes. Acta Neurol Scand 1995; 91:247–250. 26. Ansari KA. Olfaction in multiple sclerosis. With a note on the discrepancy between optic and olfactory involvement. Eur Neurol 1976; 14:138–145. 27. Doty RL, Shaman P, Dann M. Development of the University of Pennsylvania Smell Identification Test: a standardized microencapsulated test of olfactory function. Physiol Behav 1984; 32:489–502. 28. Constantinescu CS, Raps EC, Cohen JA, West SE, Doty RL. Olfactory disturbances as the initial or most prominent symptom of multiple sclerosis. J Neurol Neurosurg Psychiatry 1994; 57:1011–1012. 29. Doty RL, Li C, Mannon LJ, Yousem DM. Olfactory dysfunction in multiple sclerosis. N Engl J Med 1997; 336:1918–1919. 30. Doty RL, Li C, Mannon LJ, Yousem DM. Olfactory dysfunction in multiple sclerosis: relation to plaque load in inferior frontal and temporal lobes. Ann N Y Acad Sci 1998; 855:781–786. 31. Doty RL, Li C, Mannon LJ, Yousem DM. Olfactory dysfunction in multiple sclerosis: relation to longitudinal changes in plaque numbers in central olfactory structures. Neurology 1999; 53:880–882. 32. Elian M. Olfactory impairment in motor neuron disease: a pilot study. J Neurol Neurosurg Psychiatry 1991; 54:927–928. 33. Sajjadian A, Doty RL, Gutnick DN, Chirurgi RJ, Sivak M, Perl D. Olfactory dysfunction in amyotrophic lateral sclerosis. Neurodegeneration 1994; 3:153–157. 34. Hawkes CH, Shephard BC, Geddes JF, Body GD, Martin JE. Olfactory disorder in motor neuron disease. Exp Neurol 1998; 150:248–253. 35. Santorelli G, Marotta A. La sogli olfattometrica dell’. La sogli olfattometrica dell’epilettico in condizioni di base e dopa crisi 1964; 32:185–190.

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36. Martinez BA, Cain WS, de Wijk RA, Spencer DD, Novelly RA, Sass KJ. Olfactory functioning before and after temporal lobe resection for intractable seizures. Neuropsychology 1993; 7:351–363. 37. Hummel T, Pauli E, Schuler P, Kettenmann B, Stefan H, Kobal G. Chemosensory eventrelated potentials in patients with temporal lobe epilepsy. Epilepsia 1995; 36:79–85. 38. Moberg PJ, Doty RL, Turetsky BI, et al. Olfactory identification deficits in schizophrenia: correlation with duration of illness. Am J Psychiatry 1997; 154:1016–1018. 39. Koss E, Weiffenbach JM, Haxby JV, Friedland RP. Olfactory detection and recognition in Alzheimer’s disease [letter]. Lancet 1987; 1:622. 40. Rousseaux M, Muller P, Gahide I, Mottin Y, Roman M. Disorders of smell, taste, and food intake in a patient with a dorsomedial thalamic infarct. Stroke 1996; 27:2328–2330. 41. Rausch R, Serafetinides EA. Specific alteration of olfactory function in humans with temporal lobe lesions. Nature 1975; 225:557–558. 42. Deems DA, Doty RL, Settle RG, et al. Smell and taste disorders, a study of 750 patients from the University of Pennsylvania Smell and Taste Center. Arch Otolaryngol Head Neck Surg 1991; 117:519–528.

17 Taste Changes in the Elderly Natasha Mirza Department of Otolaryngology—Head and Neck Surgery, University of Pennsylvania Health System and Philadelphia VA Medical Center, Philadelphia, Pennsylvania, U.S.A.

INTRODUCTION The goal of the chemosensory system is the detection and discrimination of foods and the initiation and sustenance of ingestion. This system is developed in both vertebrates and invertebrates and is present even in the newborn infant. With aging, there is a decline in chemosensory functions and while extensive research has conclusively shown a decline in the olfactory system, the basis and extent of taste loss is poorly understood. It is estimated that more than 2 million Americans have some kind of chemosensory disorder (1). Taste function in the elderly is rarely if ever lost completely because of the redundancy in the mediation of taste, which occurs through several nerves. Many confounding influences also come into play in older individuals like the effects of medications, smoking, systemic illnesses, and decrease in cognitive functions. What is proven so far has been that a loss or alteration of taste affects nutritional intake in the elderly, in turn leading to multiple nutrition-related medical problems (2). With a growing geriatric population in the country, it is important to identify some of the effects of taste dysfunction and identify means of reducing them. This chapter will briefly review the anatomy and physiology of taste including taste transduction, and discuss some of the relevant research on aging and taste. Methods to help decrease some of the harmful effects of taste loss on both nutrition and quality of life will also be discussed.

DEVELOPMENT OF THE TASTE SYSTEM Morphological evidence shows that there is a functional taste system present in utero. Even premature infants respond pleasurably to sucrose and glucose. The hedonic system of pleasantness is also well developed in infants (3). The acceptance of sweet and rejection of bitter seem to be hard-wired while the effect associated with odors depends much more on experience. 195

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ANATOMY OF THE TASTE SYSTEM In mammals, taste buds are located throughout the oral cavity, in the pharynx, the laryngeal epiglottis, and at the entrance of the esophagus. Taste buds on the dorsal lingual epithelium are the most numerous (approximately 4600 per tongue) and are contained within four major classes of papillae: the fungiform, foliate, circumvallate, and the non-gustatory filiform papillae (Fig. 1). Fungiform papillae are located on the most anterior part of the tongue and generally contain one to several taste buds per papilla (4,5). They are innervated by the chorda tympani branch of the facial (VIIth cranial) nerve and appear as red spots on the tongue because they are richly supplied with blood vessels. The total number of fungiform papillae per human tongue is around 200. Foliate papillae are situated on the edge of the tongue slightly anterior of the circumvallate line. They are predominantly sensitive to sour tastes, and are innervated by the glossopharyngeal (IXth cranial) nerve and average five foliate papillae per side of the tongue with approximately 120 taste buds per foliate papilla. Circumvallate papillae are sunken papillae, with a trough separating them from the surrounding wall. The taste buds are in tiers within the trough of the papillae. They are situated on the circumvallate line and confer a sour/bitter sensitivity to the posterior one-third of the tongue. They are innervated by the glossopharyngeal (IXth cranial) nerve. There are 3 to 13 circumvallate papillae per tongue with about 250 taste buds

Figure 1 Tongue showing the distribution of the lingual papillae.

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Figure 2 Anatomy of a taste bud.

per papilla. Filiform papillae are mechanical and non-gustatory. In addition, there are 2500 taste buds on the epiglottis, soft palate, laryngeal, and oral pharynx. In mammals, taste buds are aggregations of 30 to 100 individual elongated ‘‘neuroepithelial’’ cells (50 mm in height, 30–70 mm in width), which are embedded in the lingual papillae (6). At the apex of the taste bud, microvillar processes protrude through a small opening, the taste pore, (4–10 mm in diameter) into the oral milieu. Just below the taste bud apex, taste cells are joined by tight junctional complexes. Taste cells are bipolar cells, which connect to the epithelial surface of the oral cavity via dendritic processes and to a nerve axon at the base, which invades the bud and ramifies extensively, with each fiber typically synapsing with multiple receptor cells within the taste bud (Fig. 2). The lifespan of a mammalian taste cell is approximately 10 days. As a cell ages a nerve terminal detaches, finds a developing cell, and forms new synapses. The new cell has to be of the same taste specificity as the old dying cell; in other words, taste receptor cells act as surface markers to guide nerve fibers to the right cellular target. Cells in each taste bud contain the sensory receptor cells, supporting cells, and basal cells, which differentiate into new receptor cells. A single taste bud is all that is needed to provide sensation for all the four tastes so that even the decrease in number of lingual papillae does not necessarily lead to a significant loss of taste. TASTE TRANSDUCTION It is important to understand some of the basic science that is involved in taste transduction to understand the effects of aging on this mechanism (7). Taste

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receptors trigger transduction cascades, which then activate synapses and cause the excitation of nerve fibers. A signal is produced, which is then carried to the brain relaying information on the identity and intensity of the gustatory stimulus. Receptor proteins are located on the apical surface of cells and from there the stimulus enters the cell either via an ion channel, as it does for the salty and sour tastes or via second messenger systems like cyclic adenosine monophosphate (cAMP) for bitter and sweet. Once the stimulant enters the cell, there is a change in the internal electrical state of the cell, which leads to the secretion of a neurotransmitter and activation of a nerve fiber. Each taste serves a specific purpose in the maintenance of health of an individual. Salt taste guides the intake of sodium chloride and thus the salt–water homeostasis. Sour helps with the recognition of complex foods. At low concentrations, it is pleasant but once it reaches high concentrations it becomes an unpleasant taste and leads to avoidance. It guides acid–base regulation and is sensitive to extracellular pH changes. The sweet receptor is a classical proteinaceous molecule analogous to an olfactory receptor. It responds to soluble carbohydrates in the oral cavity and regulates caloric intake, nutrition, and energy balance. It uses a second messenger like cAMP to activate a cyclic nucleotide cascade. Sweet taste has a strong hedonic effect and is well developed in infants. Bitter taste like sour is bearable and even to some extent pleasant in low concentrations but is repulsive when strong. Bitter receptors belong to the second messenger system of G proteins. They set off a G protein-coupled receptor-mediated signal, which leads to dual signaling of cAMP and cyclic guanosine monophosphate (cGMP) within the cell. Bitter taste serves as a warning system designed by nature to protect against the ingestion of harmful compounds. Again even newborn infants are seen to respond to bitter compounds like quinine with distaste. Umami as a taste was discovered about 100 years ago and is somewhat similar to sweet taste. It is derived from L-glutamate and is the taste present in monosodium glutamate (MSG), chicken broth, meat extracts, and aging cheese. It guides the intake of peptides and proteins and the receptor contains GluR4.

INNERVATION OF THE TONGUE Taste receptor cells do not have an axon. Information is relayed onto terminals of sensory fibers by transmitter. These fibers arise from the ganglion cells of three cranial nerves. Over the anterior tongue, the chorda tympani (a branch of the facial nerve) is sensory for taste and the lingual nerve, a branch of the trigeminal nerve, serves general sensation. Over the posterior tongue, taste is conveyed via the glossopharyngeal with some branches of the vagus providing additional taste sensation. In this region of the tongue, general sensation is also provided by the same two nerves. The trigeminal nerve is responsible for detecting the burning sensation caused by certain foods, e.g., peppers. Taste nerves normally inhibit one another across the midline. Therefore, when a nerve is damaged there is a decrease in inhibitory impulses from that side which in turn leads to an increase in responses from the opposite side. This disinhibition of the damaged nerve leads to an overall preservation of taste and total ageusia or loss of taste is very rare. On the other hand, the olfactory system depends on one nerve and is therefore more vulnerable to injury.

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Central Pathways Primary gustatory fibers synapse centrally in the medulla (in a thin line of cells called the nucleus of the solitary tract). From there the information is relayed to the somatosensory cortex for the conscious perception of taste and to the hypothalamus, amygdala, and insula, giving the so-called ‘‘affective’’ component of taste (1,2). This is responsible for the behavioral response, e.g., aversion, gastric secretion, and feeding behavior.

REVIEW OF LITERATURE The influence of aging on taste has been investigated in a large number of studies. Verification of taste loss is an important aspect of testing when a patient comes to the doctor complaining of taste loss. It is often found that the sense of taste may be normal but the sense of smell is impaired leading to a loss of flavor. Flavor is a complete sensory experience arising from ingested stimulant molecules and involves taste, smell, texture, and temperature. A review of more than 30 papers on this topic dating from 1959 on young and elderly subjects was conducted. Almost all the studies used similar stimulants, namely sodium chloride (NaCl) for salty, sucrose for sweet, acetic or citric acid for sour, and quinine sulfate for bitter. The type and extent of taste loss over the decades and which gender was most affected emerged as fairly inconsistent. Taste studies looked at two primary aspects of taste: acuity and intensity. Taste acuity measures two different parameters: a. taste detection or threshold testing, which is a measure of the concentration at which a distinction can be made between a taste substance (tastant) and water; and b. taste recognition, i.e., which is sweet, sour, salty, or bitter. Taste intensity is a suprathreshold measure of taste qualities. Whole mouth testing is used to assess the individual’s ability to detect, identify, and evaluate the intensity of different concentrations of the four taste substances. Spatial taste testing is used to assess different areas of the tongue to identify if there is localized impairment of taste. At the Smell and Taste Center at the University of Pennsylvania, a drop of the tastant (sweet, sour, salt, and bitter) is placed on the tongue with a pipette and the subject is asked to identify the solution, to rate the confidence of their answer and also to rate the intensity of the solution on a nine-level scale of increasing shades of gray. In electrogustometry, a weak electrical current is delivered to the various taste regions in the oral cavity (8). Most of the studies reviewed have discovered that with increasing age there is an increase in taste thresholds for all the four tastes (9). Cooper et al. (10) in 1959 used whole mouth testing and found that taste sensitivities remained stable till the age of 50 and after that there was an increase in all taste thresholds. In 1976, Murphy and Gilmore (11) found that taste thresholds were increased sevenfold in older individuals. In 1986, Bartoshuk et al. (12) used magnitude matching (comparing taste intensity to loudness intensity) and had similar results. In 1988, Lasilla et al. (13) found that the elderly had significantly higher identification and detection thresholds for all four tastants.

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Several other studies found that the main effect of aging was on the salty taste (14,15). In 1979, Grzegorczyk et al. (16) found that there was a statistically significant increase in salt detection thresholds from the second to ninth decades of life. In 1982, Weiffenbach et al. (17) determined taste thresholds for all the four tastants using forced choice trials. They found a significant increase in detection thresholds for sodium chloride and a slight increase for quinine sulfate. Later in 1986, Weiffenbach et al. (18) used the direct scaling method (matched the linear extent of a tape to the perceived intensity of the stimulus) and found that there was a decrease in taste sensitivities for sodium chloride, quinine, and citric acid. Similar results were obtained by Spitzer (19) and Nordin et al. (20). In 2001, Mojet et al. (21), using the ascending staircase method of tastant identification, found that taste sensitivity was maximally affected in the elderly for NaCl and umami and least for sucrose. Osada et al. (22) and Bradley (23) in separate studies recorded the neuropsychological responses from the chorda tympani of young and old rats and found that responses to NaCl decreased significantly with aging. Sweet taste in most of the studies reviewed seemed the most robust and survived the longest (24). Most studies have also found that males were much more vulnerable to the effects of aging compared with age-matched females both for taste thresholds and intensities. Fungiform papillae are concentrated on the anterior tip of the tongue and anterior lateral margins in humans, and it has been demonstrated that NaCl threshold is inversely related to the number of fungiform papillae (more papillae ¼ more sensitivity, lower threshold) (25). Miller (26) in 1988 studied cadaveric tongues and looked at taste bud densities (the number of taste buds per surface area in cm2). Miller looked at three age groups and found no differences in the mean taste bud densities across the age groups. There was a decrease in the thickness of the upper layer of the epithelium and a flattening of papillae with advancing age, and such changes did influence the number of fungiform papillae, but there was no proof of changes in taste receptor distribution or function. On the other hand, Arey et al. (27) found degenerative changes in taste receptors with a regression of gustatory papillae, a decrease in the number of papillae with age, and a decrease in the number of taste buds. Arey et al. found more than 250 taste buds in each circumvallate papilla in the young adult and less than 100 in persons over 70 years. Bradley found no significant change in the numbers of taste buds on the fungiform papillae of rats versus monkeys versus humans. However, the taste buds in the elderly were functionally impaired as indicated by electrophysiologic responses recorded form the chorda tympani. Bradley also suggested that there may be changes in the central nervous system (CNS) responses to these electrophysiologic stimuli in the aged. Drawbacks in the studies reviewed were the lack of concordance in the various studies, procedural differences, and different psychophysical stimuli presented. Sample sizes were small and some studies were conducted either on cadaveric tongues or on animals and the results could not be directly related to human subjects.

REASONS FOR LOSS OF TASTE IN THE ELDERLY Besides the loss of taste papillae and taste bud numbers and function discussed above, some of the other factors which lead to changes in taste perception in the aged population include the following:

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a. Decrease in the flow of saliva, which is also of increased salt concentration and increased viscosity. This in turn leads to decreased taste receptor stimulation. b. An increase in the incidence of gingivitis and periodontal disease in this population. This has been proven to increase taste thresholds. Also several studies have shown a lowering of taste thresholds with an improvement in oral hygiene. Trials of antifungal troches and antibiotics have also been tried in an effort to improve oral health. c. The use of dentures covers part of the soft palate where there are taste receptors, thereby decreasing taste. d. Systemic diseases like diabetes, gastric disease, hypothyroidism, liver disease, chronic renal failure, Alzheimer’s, and Parkinson’s disease. Gastric reflux is a common cause of dysgeusia and in the elderly can worsen problems with taste dysfunction. e. Use of medications, especially antihypertensive drugs and chemotherapeutic agents, affect taste. Medications are suspected of introducing tastable substances into the blood and thereby altering taste thresholds. Schiffman found that certain drugs could modify taste transduction or the turnover of cells and alter taste perception. The taking of more than one medication (the so-called polypharmacy) is common among older people and this compounds the problem with taste dysfunction. f. Smoking affects taste, especially the bitter sense. Hsu and Davis found that smoking decreased gustatory sensitivity. g. The elderly require longer stimulation to integrate the sensory information further impairing taste.

EFFECTS OF TASTE LOSS IN THE ELDERLY What most elderly individuals complain of is a loss of flavor and enjoyment of foods, which is most often related to the well-known loss of olfaction combined with some taste loss (28). Added to this problem is a loss of memory and some cognitive changes and the effect is a loss of appetite in older individuals. This puts the elderly individual at higher risk for depression, anorexia, and weight loss. When elderly individuals lose their sense of taste, they often blame the food and stop eating certain foods. Foods that are essential for nutritional balance often do not taste good enough and the elderly resort to eating unhealthy foods like candies and pastries instead of meats and vegetables. The decreased consumption of proteins and other nutrients leads to loss of bone mass, weakening of the immune system, and hypertension (29). The increase in salty taste thresholds may be responsible for an increase in the intake of salt in the elderly. This is one of the causative factors contributing to the development of hypertension. Studies by Spitzer have shown that patients with hypertension demonstrate a greater preference for salt compared to younger individuals. Institutionalized individuals also show an increase in taste thresholds, which may account for the poor intake and nutritional status of some of these people. Poor taste perception combined with olfactory loss puts the elderly individual at risk for food poisoning from the ingestion of spoiled foods.

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THERAPY FOR TASTE LOSS No specific therapy or pharmacological agents are available to reverse the loss of taste with aging and in general the prognosis is poor. Awareness of taste decrement in the aged is important to make sure that these individuals get a balanced diet and prevent health problems, which are secondary to a poor and unhealthy diet. Flavor amplifiers have been developed to enhance the flavor by adding an extra ingredient to the food. Examples of these are seasoning salts, fruit juices, meat stocks, and gravy. MSG also helps as a flavor enhancer, especially for protein-based dishes because it adds a savory taste to these foods, e.g., meat loaf, casserole, etc. Other food enhancers include vinegars, sun-dried tomatoes, herbs, and spices. Color enhancers make the food more appealing and enjoyable to people who have a poor appetite. Caregivers need to be educated in food preparation to prepare a nutritionally balanced diet with the appropriate addition of additives and flavor enhancers especially when helping to prepare meals for institutionalized individuals. Awareness of the fact that gastrointestinal irritation, if any of the additives is in too great a quantity, should also be created. Anecdotal evidence also exists that lack of the trace elements can lead to decreases in metabolic functions. Chauhan et al. (30,31) have therefore suggested that zinc supplementation be tried to enhance taste along with vitamins A, B12, and folic acid for the maintenance of taste bud integrity.

CONCLUSION Current research is geared toward the development of drugs containing taste ligands, which activate or inhibit taste receptor proteins. These in turn can then increase or decrease specific tastes (e.g., artificial sweeteners like saccharin and aspartame). Awareness of the loss of taste with aging should be an important part of education both for caregivers of the elderly and for the geriatric population so that a conscious effort is made toward the preparation of a nutritionally balanced diet. When an individual notices a loss of taste a referral to a chemosensory center should be made, where a multidisciplinary team approach can help with the elimination of the reversible causes of taste loss. Protectants of taste should be researched for and avoidance of drugs and harmful habits like smoking should be encouraged, as they will help preserve the natural taste function longer. Keeping the sense of taste strong is essential for the enjoyment of life and for the survival of the human race as a whole.

ACKNOWLEDGMENT The author would like to thank Ms. Juliette Watts of the Philadelphia Veteran’s Administration Medical Media Department for her artistic help.

REFERENCES 1. Schiffman SS. Taste and smell in disease. N Engl J Med 1983; 308:1275–1279. 2. Bartoshuk LM. Taste. Robust across the age span. Ann NY Acad Sci 1989; 561:65–75.

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3. Temple EC, Hutchison I, Laing DG, Jinks AL. Taste development: differential growth rates of tongue regions in humans. Dev Brain Res 2002; 135:65–70. 4. Satoh Y, Seluk LW. Taste threshold. Anatomical form of fungiform papillae and aging in humans. J Nihon Univ Sch Dent 1988; 30:22–29. 5. Sogovia C, Hutchison I, Laing DG, Jinks AL. A quantitative study of fungiform papillae and taste pore density in adults and children. Dev Brain Res 2002; 138:135–146. 6. Nelson GM. Biology of taste buds and the clinical problem of taste loss. Anat Rec (New Anat) 1988; 253(3):70–78. 7. Lindemann B. Receptors and transduction in taste. Nature 2001; 413:219–225. 8. Miller SL, Mirza N, Doty RL. Electrogustomertric thresholds: relationship to anterior tongue locus, area of stimulation, and number of fungiform papillae. Physiol Behav 2002; 75:753–757. 9. Schiffman SS, Hornack K, Reilly D. Increased taste thresholds of amino acids with age. Am J Clin Nutrition 1979; 32:1622–1627. 10. Cooper RM, Bilash I, Zubek JP. The effect of age of taste sensitivity. J Gerontol 1959; 14(1):56–58. 11. Murphy C, Gilmore MM. Quality specific effects of aging on the human taste system. Percept Psychophys 1989; 45:121–128. 12. Bartoshuk LM, Rifkin B, Marks LE, Bars P. Taste and aging. J Gerontol 1986; 41(1): 51–57. 13. Lasilla V, Sointu M, Raiha I, Lehtonen A. Taste thresholds in the elderly. Proc Finn Dent Soc 1988; 84(5–6):306–310. 14. Matsuda T, Doty RL. Regional taste sensitivity to NaCl: relationship to subject age, tongue locus and area of stimulation. Chem Senses 1995; 20:283–290. 15. Zallen EM, Hooks LB, O’Brien K. Salt taste preferences and perceptions of elderly and young adults. J Am Dietetic Assoc 1990; 90(7):947–951. 16. Grzegorczyk PB, Jones SW, Mistretta CM. Age related differences in salt taste acuity. J Gerontol 1979; 34(6):834–840. 17. Weiffenbach JM, Baum BJ, Burghauser R. Taste thresholds: quality specific variation with human aging. J Gerontol 1982; 37(3):372–377. 18. Weiffenbach JM, Cowart BJ, Baum BJ. Taste intensity perception in aging. J Gerontol 1986; 41(4):460–468. 19. Spitzer ME. Taste acuity in institutionalized and noninstitutionalized elderly men. J Gerontol Psychol Sci 1988; 43(3):71–74. 20. Nordin S, Razani JL, Markison S, Murphy C. Age associated increases in intensity discrimination for taste. Exp Aging Res 2003; 29:371–381. 21. Mojet J, Chris-Hazelhof E, Heidema J. Taste perception with age: generic or specific losses in threshold sensitivity to the five basic taste. Chem Senses 2001; 26:845–860. 22. Osada K, Komai M, Bryant BP, Suzuki H, Sunoda K, Furukawa Y. Age related decreases in neural sensitivity to NaCl in SHR-SP. J Vet Med Sci 2003; 65(3):313–317. 23. Bradley RM. Effects of aging on the anatomy and neurophysiology of taste. Gerondontics 1988; 4(5):244–248. 24. Schiffman SS, Lindley MG, Clark TB, Makino C. Molecular mechanism of sweet taste: relationship of hydrogen bonding to taste sensitivity for both young and elderly. Neurobiol Aging 1981; 2:173–185. 25. Doty RL, Bagla R, Morgenson M, Mirza N. NaCl thresholds: relationship to anterior tongue locus, area of stimulation, and number of fungiform papillae. Physiol Behav 2001; 72:373–378. 26. Miller IJ. Human taste bud density across adult age groups. J Gerontol 1988; 43(1): B26–30. 27. Arey LB, Tremaine MJ, Monzingo FL. Numerical and topographical relations of taste buds to circumvallate papillae throughout the life span. Anat Rec 1935; 64:9–24.

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28. Deems DA, Doty RL, Settle RG, et al. Smell and taste disorders, a study of 750 patients from the University of Pennsylvania Smell and Taste Center. Arch Otolaryngol Head Neck Surg 1991; 117(5):519–528. 29. Mann NM. Management of smell and taste problems. Cleveland Clin J Med 2002; 69(4):329–336. 30. Chauhan J, Hawrysh ZJ, Gee M, Donald EA, Basu TK. Age related olfactory and taste changes and interrelationships between taste and nutrition. J Am Dietetic Assoc 1987; 87(11):1543–1550. 31. Chauhan J. Relationships between sour and salt taste perception and selected subject attributes. J Am Dietetic Assoc 1989; 89(5):652–657.

18 Sinusitis: Diagnosis, Treatment, and Complications Berrylin J. Ferguson and Umamaheswar Duvvuri Division of Sino-Nasal Disorders and Allergy, Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION Sinusitis is a major public health concern, which affects approximately 20 million people. It is one of the main complaints that leads adults of any age to seek medical assistance (1). The diagnosis of sinusitis in the geriatric population is fraught with all the difficulties faced in the diagnosis in a younger population. These include lack of specificity of symptoms for rhinosinusitis (2) as well as the fact that different pathophysiologic conditions such as allergic rhinitis, vasomotor rhinitis, viral rhinosinusitis, bacterial rhinosinusitis, and even some headaches are collectively interpreted by many patients as ‘‘sinus trouble.’’ Complicating the treatment of rhinosinusitis, regardless of cause, in the older population is the increased incidence of concurrent disease states, which increase the likelihood of adverse drug reactions or interactions with the patients’ regular medications. Sinus disease may present in either the acute or chronic forms. Acute sinusitis is defined as the persistence of symptoms for less than four weeks. Chronic sinusitis is defined as the persistence of symptoms for greater than 12 weeks. Subacute sinusitis falls between the two, lasting greater than four weeks but less than 12 weeks. Infectious causes of rhinosinusitis include viral, bacterial, and fungal etiologies and are a more common cause in acute sinusitis than chronic sinusitis. In general, chronic rhinosinusitis is less frequently due to an infectious etiology and more frequently caused by noninfectious inflammation, although there can be chronic bacterial or fungal rhinosinusitis. Chronic rhinosinusitis is frequently subdivided into chronic rhinosinusitis with polyps and chronic rhinosinusitis without polyps.

ACUTE SINUSITIS Acute sinusitis has been better studied than chronic sinusitis. The majority of cases are caused by the common cold, a viral infection caused by a variety of different viruses, each with a multitude of serotypes. While upper respiratory tract infections 205

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are far more common in children than adults, the older adult can also have a cold. One risk factor for developing a cold is exposure to sick children, even though this may be somewhat reduced with hand washing. A viral infection cannot be reliably differentiated from a bacterial infection on the basis of symptoms, physical findings, or radiographs. In general, however, most cold symptoms will improve after three days although cough may persist for many weeks. The treatment of a cold is supportive. The use of decongestants may be problematic in the older population since decongestants may aggravate urinary retention from prostatic hypertrophy, exacerbate cardiac arrhythmias, or raise blood pressure. All of these comorbidities are more common in the older patient. Saline nasal sprays, mucolytics (such as increased hydration and high-dose guaifenesin), and chicken soup can be prescribed without fear of causing harm. Although analgesics such as nonsteroidal anti-inflammatory drugs can provide some symptomatic relief, caution should be utilized in patients taking anticoagulants or with a history of gastritis. Diagnosis Symptoms associated with acute sinusitis include facial pressure, pain of the maxillary teeth, postnasal drip, colored nasal discharge, and sometimes headaches. Facial pain or headache in the absence of other symptoms is rarely caused by sinusitis. Maxillary tooth pain is the most specific sign of bacterial sinusitis but is present in <15% of cases (3). Presence of unilateral purulent drainage is also more common in acute bacterial sinusitis; however, whether this is true for the geriatric population is unknown. Other more uncommon and nonspecific symptoms include halitosis, fever, nasal obstruction, cough, or otologic problems. In the elderly person who lacks dentition, a complaint of upper gum pain may be caused by maxillary sinusitis. Dental infection of the maxillary teeth can also lead to maxillary sinusitis. The pathogens of odontogenic sinusitis are more likely to consist of mixed microbial flora and include anaerobes. The pertinent history should detail the severity of the complaints, precipitating and alleviating factors, associated symptoms, and a thorough past medical history. Obtaining a thorough past medical history and a list of medications, both prescription and over the counter, is particularly important in the geriatric patient. This allows the physician to evaluate for drug interactions and conditions that might mimic some feature(s) of sinusitis. The physical examination by the otolaryngologist should consist of visualization of the nasal cavity. If the nose is so swollen that it cannot be adequately examined then it should be decongested with topical decongestants sprays or the placement of cotton pledgets impregnated with vasoconstrictors such as oxymetazoline or a few drops of 1:1000 epinephrine combined with 4% lidocaine. Local anesthetic of cocaine has fallen out of favor because of medical legal implications (i.e., patient drives home from the exam and has an accident in which the drug screen would be positive from the cocaine use in the exam). In addition, cocaine is associated with increased cardiac toxicity. Frequently, a culture of purulent material from the middle meatus can be reliably obtained by a Calgi swab or aspirate. The correlation of endoscopically obtained bacteriologic findings is 70% to 90% with those obtained by maxillary puncture (4). Examination of the nasal cavity and acquisition of a culture is facilitated with the use of a rigid endoscope. The location of the purulent material in the nasal cavity correlates with the associated sinuses. Therefore, purulence in the

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middle meatus is associated with maxillary, anterior ethmoid, or frontal sinusitis. Pus streaming over the posterior superior aspect of the middle turbinate and above the eustachian tube orifice would indicate a posterior ethmoid sinusitis, while purulence coming from above the choana, 30
from the floor of the nose (the location of the sphenoid recess), would indicate a sphenoid sinusitis. A history of prior sinus surgery should be noted and may facilitate the aspiration of culture material. Cultures should be obtained using proper technique, which includes special care in avoiding contamination with the anterior nasal vestibule. The presence of nasal polyps, vestibulitis, septal excoriation, turbinate hypertrophy, or evidence of prior surgery, scarring, and the character of the mucosa should all be noted during examination. The use of a 30
endoscope allows for improved visualization of the maxillary sinus ostia, and possibly the sinus contents, if the ostium is widely patent. If the patient has had prior sinus surgery, a flexible fiber-optic scope can be introduced into the maxillary sinus for visualization of the floor and lateral aspects of the sinus. Occasionally, purulent secretions will be seen in the floor of the maxillary sinus even when the nasal cavity examination is normal. Plain radiographs are usually obtained only if a maxillary puncture is required and there is a need to confirm presence of disease. An unenhanced coronal sinus computed tomography (CT) should be performed before surgical intervention. Another indication for sinus CT is the patient with persistent or puzzling symptoms despite appropriate treatment.

ANTIBIOTIC SELECTION If the patient has symptoms that are worsening after three or four days or not improving after a week then clinically a bacterial sinusitis should be suspected. There is no information on whether the pathogens responsible for sinusitis in the older population differ in species or in antimicrobial resistance patterns from those found in younger adults. The 2004 sinus and allergy health partnership guideline recommendations for antimicrobial therapy in adults with mild symptoms include amoxicillin/clavulanate (1.75–4 g/250 mg/day), amoxicillin (1.5–4 g/day), cefpodoxime proxetil, cefuroxime axetil, or cefdinir. Trimethoprim/sulfamethoxazole (TMP/ SMX) (Bactrim or Septra), doxycycline, azithromycin, clarithromycin, erythromycin, or telithromycin may be considered in patients with b-lactam allergies, although bacteriologic failure rates of 20% to 25% may be encountered if treatment is not directed by culture and sensitivities. The recent guidelines are slightly different for adults with mild disease who have received antibiotics in the previous four to six weeks or for adults with moderate disease and include the following recommendations: respiratory fluoroquinolones (e.g., moxifloxacin, gatifloxacin, and levofloxacin, listed in order of potency) or high-dose amoxicillin/clavulanate (4 g/250 mg/day). The widespread use of respiratory fluoroquinolones for patients with milder disease may promote resistance to this broad-spectrum class of antibiotics and should be discouraged. The fluoroquinolones may, depending on the drug in the class, cause more disequilibrium in the geriatric age group compared to younger adults. Ceftriaxone (parenteral, 1–2 g/day  5 days) or combination therapy with adequate grampositive and -negative coverage is also an alternative. Examples of appropriate regimens of combination therapy include high-dose amoxicillin or clindamycin plus cefixime, or high-dose amoxicillin or clindamycin plus rifampin. Rifampin should not be used as monotherapy, or longer than 10–14 days as resistance to this agent quickly develops. Failure of a patient to respond to antimicrobial therapy after

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72 hours of therapy should prompt either a switch to alternate antimicrobial therapy or re-evaluation of the patient (5). If sinusitis is of odontogenic origin, then antibiotic coverage of anaerobes and oral flora is appropriate and includes penicillin and clindamycin. Antibiotics appropriate for sinusitis with their cautions/contraindications and drug interactions pertinent to the elderly are listed in Table 1. This table is not all-inclusive, particularly with regard to antibiotics undergoing hepatic metabolism. IV antibiotics can be helpful in patients whose bacterial organism is resistant to oral antibiotics or in those with impaired host defenses and vascular insufficiency (e.g., diabetics) who may benefit from the higher antibiotic serum levels, which can be obtained intravenously. Laboratory monitoring of liver, renal, and hematological parameters depend on the antimicrobial agent selected. In general, patients taking warfarin should have their international normalized ratio (INR) monitored while on any oral antibiotic, since risk of bleeding is increased

Table 1 Oral Antibiotics Appropriate for Acute Bacterial Sinusitis with Cautions/ Contraindications and Drug Interactions Pertinent to the Elderly Possible drug interactions Fluoroquinolones (moxifloxacin, gatifloxacin, levofloxacin) Amoxicillin/clavulanate

Possible with a number of antiarrhythmics and antihypertensives, list extensive Allopurinol, methotrexate

Amoxicillin

Allopurinol, methotrexate

Ceftriaxone

Oral cephalosporins

Macrolide

Clindamycin

Trimethoprim sulfamethoxazole Tetracycline

Rifampin

Antacids and proton pump inhibitors may decrease absorption Numerous with other medications undergoing hepatic metabolism Neuromuscular blockers

Digoxin, MAO 2 inhibitors, methotrexate, phenytoins, warfarin Numerous with other medications undergoing hepatic metabolism Numerous with other medications undergoing hepatic metabolism

Carry a caution for use in elderly patients Yes Increased incidence of disequilibrium No but caution if impaired liver function or creatinine clearance <30 No but caution if impaired renal function No but caution if renal dysfunction or hyperbilirubinemia No

No but caution if impaired liver function No but caution if impaired liver or renal function or ulcerative colitis or history of antibioticassociated colitis Yes; also caution if bone marrow suppressed or if impaired liver function No but caution if impaired renal or liver function No but caution if impaired liver function; hepatic enzyme inducer

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secondary to altered vitamin K production by gut flora. Antibiotics increase risk for Clostridia difficile overgrowth in the gut. Many practitioners recommend that while the patient is taking oral antibiotics, yogurt, Lactobacillus, or probiotic administration be encouraged concurrent with the antibiotic (6). In addition, selection of an antibiotic with the narrowest spectrum targeted to the causative organism will reduce impact on the native microflora. Fungal Sinusitis Acute invasive fungal sinusitis should be suspected in the immunocompromised patient with objective evidence of sinus disease. Radiographs in invasive fungal sinusitis are indistinguishable from bacterial sinusitis and show no evidence of bony erosion until the disease is far advanced. An endoscopic culture or maxillary sinus puncture with lavage and culture should be obtained unless the latter is contraindicated because of coagulopathy. If the patient has poorly controlled diabetes, then mucormycosis should be suspected. The earliest symptom in mucormycosis is commonly anesthesia of the affected area followed by blackening of the mucosa (7). If invasive fungal sinusitis is suspected, a frozen section biopsy of the suspected tissue or immediate fungal stain of an aspirate along with culture should be obtained. If positive for invasive fungal sinusitis, then every effort should be made to reverse the patient’s source of immunocompromise, and systemic antifungals should be selected appropriate to the fungus cultured or suspected histopathologically. Surgery should be conservative with debridement of necrotic areas. Acute invasive fungal rhinosinusitis may also be caused by a wide variety of fungi in addition to mucormycosis, with the most common being Aspergillus fumigatus, particularly in the nondiabetic immunocompromised patient.

CHRONIC SINUSITIS Stankiewicz and Chow (2) looked prospectively at the relationship between the definition of chronic rhinosinusitis and the diagnosis of bacterial chronic rhinosinusitis in 78 patients ranging in age from 16 to 78. Patients who had a strong history of sinusitis by symptom score underwent same-day endoscopy and CT of the sinuses. Of those patients who had no polyps or purulence endoscopically, over half had normal CT scans. Moreover, there was no difference in symptom scores between CT(þ) and CT(–) patients. Eighty percent of normal CT scans were associated with a normal nasal endoscopy (8). Thus, patients with symptoms suggestive of chronic sinusitis will have no objective evidence of sinusitis half of the time. Nasal endoscopy as the primary intervention, with only those patients with positive findings undergoing treatment, was calculated to be more cost effective than obtaining a CT scan on all patients and treating only those with disease or empirically treating all patients with broad-spectrum antibiotic and nasal steroid sprays for one month and only obtaining a CT scan on the failures (9). Chronic sinus disease may be caused by bacteria and fungi or be independent of an identifiable infectious agent. The bacteria commonly implicated in chronic sinsusitis are Staphylococcus aureus and gram-negatives (such as Pseudomonas aeruginosa) in patients who have had prior surgery or have impaired mucosal defenses. The role of anaerobes and coagulase negative staphylococcus in chronic sinusitis is controversial (1). Fungi can elicit an allergic reaction causing allergic fungal

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sinusitis. Allergic fungal sinusitis is far more common in younger people but has been reported to occur in patients in their 70s (10). Diagnosis of chronic sinusitis requires objective evidence of sinus disease for at least three months. It is useful to divide patients into those with nasal polyps and those without. Patients with bilateral nasal polyposis are less likely to have a primary bacterial infection. If a bacterial pathogen is identified, then it should be treated with a culture-directed antibiotic. The course of therapy recommended is longer than that in acute bacterial sinusitis. A useful guideline is to continue the antibiotic for several days beyond symptom resolution or when symptom improvement plateaus. There are no controlled studies on the efficacy of antimicrobial therapy in chronic sinusitis. Nasal steroid sprays are a cornerstone of medical therapy in patients with chronic sinusitis, particularly in the patient with nasal polyps, but are frequently used in any patient with symptoms of nasal congestion or postnasal drainage. The use of topical steroids reduces mucosal inflammation. Most topical steroid sprays have low systemic absorption and pose little risk of systemic steroid side effects. Although Beclomethasone nasal spray has been reported to cause increased intraocular pressure, this has not been reported in nasal sprays with less systemic bioavailability (11). There are no studies to date regarding the effect of nasal steroid sprays on ocular pressures in the elderly. Systemic steroid use is potentially more hazardous in the geriatric patient for the following reasons: glucose control may be compromised in diabetic patients, osteopenia/osteoporosis may be exacerbated, and steroids may affect blood pressure control and the progression of cataracts. The majority of patients with sinus symptoms severe enough to require endoscopic sinus surgery are allergic. Whether this is true in the geriatric population is unknown. Allergies usually decrease with age but may be present even through the ninth decade. In general, first-generation sedating antihistamines are not suitable in elderly patients because of sedation and anticholinergic side effects.

CHRONIC FUNGAL RHINOSINUSITIS Fungus Balls Fungus balls of the paranasal sinuses comprise the most common chronic form of fungal sinusitis and are also a noninvasive form. Older individuals may be more susceptible although there are no definitive data to substantiate this (12–14). Ferreiro et al. (15) report a mean age of 64 years. There does appear to be a female predominance. Associated medical conditions such as allergic rhinitis and aspirin sensitive asthma are no higher than the general population. The clinical presentation of fungus balls is similar to that of chronic rhinosinusitis. Common symptoms include nasal obstruction, facial pain, and purulent discharge. Eighteen percent of patients were asymptomatic in the largest review and diagnosis was made incidentally on radiographs (14). The most commonly affected sinus is the maxillary followed by the sphenoid. Frontal sinus involvement is uncommon. Ethmoid sinus involvement is frequently contiguous with the maxillary. The treatment of fungus balls of the sinus consists of surgical removal. With the advent of endoscopic instrumentation, most fungus balls can be approached endoscopically. Irrigation is also helpful in the debriding process. The management of the asymptomatic patients remains controversial and removal is often suggested to prove the diagnosis. Surgical resection may be advocated if the fungus ball is thought to precipitate asthma or if bony erosion is present on radiographs. If the

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asymptomatic patient becomes immunocompromised, the fungus ball may become invasive. Patients with a possible fungus ball, facing expected immunocompromise because of bone marrow transplant, for example, should have the fungus ball removed prior to becoming immunocompromised.

CHRONIC INVASIVE FUNGAL SINUSITIS Chronic invasive fungal sinusitis is a rare entity that has been proposed by Stringer and Ryan (15) to describe nonfulminant invasive fungal disease in immunologically intact patients. In a case series of four patients, Stringer and Ryan reported a geriatric predominance, with three-fourths of patients being greater than 65 years of age. Symptoms directly related to the invasive disease may take several months to manifest and may only be apparent after the orbit or skull base is involved. Mucosal thickening is often noted, although signs of bony erosion or obliteration of fat planes around the maxillary sinus are also associated with invasive fungal disease. Chronic invasive fungal rhinosinusitis may mimic many other disorders including benign and/or malignant neoplasms, Wegener’s granulomatosis, lymphoma, allergic fungal rhinosinusitis, and rhinoscleroma. Chronic invasive fungal rhinosinusitis has specifically been associated with Mucor, Alternaria, Curvularia, Bipolaris, Candida, Drechslera, Sporothrix, and Pseudallescheria. Current treatment of chronic invasive fungal sinusitis consists of surgical debridement with adjuvant antifungal therapy. There is no consensus as to the extent of surgical debridement necessary to treat the disease, and recommendations have ranged from wide aeration of the diseased sinuses to a complete extirpation of the diseased tissue. It is also generally recommended that serial CT scans and nasal endoscopy be performed every three to four months after completion of therapy. Recurrent disease should be promptly addressed with further surgery and/or antifungal therapy. Special Considerations in the Surgical Management of the Geriatric Patient Geriatric patients with significant comorbidities require preoperative medical evaluation to minimize potential anesthetic and perioperative complications. In particular, perioperative and postoperative hypertension may increase the potential for epistasis and necessitate nasal packing. The geriatric male patient is more likely to have prostatic hypertrophy and suffer from postanesthetic urinary retention. Before discharge from the outpatient surgical suite all patients must be able to void on their own. Beyond this, sinus surgery is similar to that performed in the younger population. Ramadan and VanMetre (16) compared endoscopic sinus surgeries (ESSs) in 46 geriatric patients (>65 years) to 522 younger patients (18–64 years). Approximately 33% of older patients required frontal sinusotomy as primary surgery compared to 21% of younger patients. Sphenoidotomy as either a primary or revision surgery was twice as common in the geriatric patient (68%) compared to the younger group. Revision ESS carried a higher risk of orbital injury (not blindness) (27% vs. 3%), and hemorrhage (18% vs. 3.4%) in geriatric patients compared to younger patients. There was no difference in complications between the two groups for primary surgery. None of the geriatric patients had a cerebrospinal fluid (CSF) leak compared to 2% in the younger group.

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SUMMARY Diagnosis of acute, subacute, or chronic sinusitis in the geriatric population must be based on a full history and physical examination, which includes rigid nasal endoscopy. Endoscopically guided cultures and appropriate use of CT scans may be helpful in guiding therapy. Special considerations regarding the use of antibiotics in the older adult include the presence of comorbidities, potential adverse effects, and interaction with other medications, as well as status of liver and renal function. Overall, sphenoid surgery is more common in the geriatric patient and there is a slight increase in minor complications with revision surgery.

REFERENCES 1. Benninger MS, Ferguson BJ, Hadley JA, et al. Adult chronic rhinosinusitis: definitions, diagnosis, epidemiology, and pathophysiology. Otolaryngol Head Neck Surg 2003; S129:S1–32. 2. Stankiewicz JA, Chow JM. A diagnostic dilemma for chronic rhinosinusitis: definition, accuracy and validity. Am J Rhinol 2002; 16:199–202. 3. Anon J, Jacobs MR, Poole MD, et al. Antimicrobial treatment guidelines for acute bacterial rhinosinusitis. Otolaryngol Head Neck Surg 2004; S130(1):S1–S45. 4. Williams JW Jr, Simel DL. Does this patient have sinusitis? Diagnosing acute sinusitis by history and physical examination. JAMA 1993; 270(10):1242–1246. 5. Talbot GH, Kennedy DW, Scheld WM, Granito K. Rigid nasal endoscopy versus sinus puncture and aspiration for microbiologic documentation of acute bacterial maxillary sinusitis. Clin Infect Dis 2001; 33:1668–1675. 6. Bergogne-Berezin E. Treatment and prevention of antibiotic associated diarrhea. Int J Antimicrob Agents 2000; 16(4):521–526. 7. Ferguson BJ. Mucomycosis of the nose and paranasal sinuses. Otolaryngol Clin North Am Fungal Rhinosinusitis Spectr Dis 2000; 33(2):349–365. 8. Stankiewicz JA, Chow JM. Nasal endoscopy and the definition/diagnosis of chronic rhinosinusitis. Otolaryngol Head Neck Surg 2002; 126:623–627. 9. Stankiewicz JA, Chow JM. Cost analysis in the diagnosis of chronic rhinosinusitis. Am J Rhinol 2003; 12:139–142. 10. Ferguson BJ. Eosinophilic mucin rhinosinusitis: a distinct clinicopathological entity. Laryngoscope 2000; 110(5 Pt 1):799–813. 11. Opatowsky I, Feldman RM, Gross R, Feldman ST. Intraocular pressure elevation associated with inhalation and nasal corticosteroids. Ophthalmology 1995; 102:177–179. 12. de Shazo RD, O’Brien M, Chapkin K, et al. Criteria for the diagnosis of sinus mycetoma. J Allergy Clin Immunol 1997; 99:475–485. 13. Ferreiro JA, Carlson BA, Cody DT. Paranasal sinus fungus balls. Head Neck 1997; 19: 481–486. 14. Klossek JM, Serrano E, Peloquin L, Percodani J, Fontanel JP, Pessey JJ. Functional endoscopic sinus surgery and 109 mycetomas of paranasal sinuses. Laryngoscope 1997; 107:112–117. 15. Stringer SP, Ryan MW. Chronic invasive fungal rhinosinusitis. Otolaryngol Clin North Am 2000; 33(2):375–387. 16. Ramadan HH, VanMetre R. Endoscopic sinus surgery in geriatric population. American Rhinologic Society meeting, Nashville, Tn., May 2003.

19 Allergic Rhinitis in the Elderly Stephen J. Chadwick Department of Otolaryngology, Southern Illinois School of Medicine, Springfield, and ENTA Allergy Head and Neck Institute, Decatur, Illinois, U.S.A.

‘‘Aging is a process that converts healthy adults into frail ones with diminished reserves in most physiologic systems and an exponentially increasing vulnerability to most diseases and to death’’ (1). With improving American longevity and the maturing of the ‘‘Baby Boomers’’ (born in the 1940s and 1950s), health-related demands of the geriatric population continue to increase. Allergic rhinitis is common and increasing in this population. Some of this increase is related to pollutants and irritants as ‘‘ . . . allergically predisposed parents are producing allergically predisposed progeny in an increasingly antigenic and irritant-laden environment’’ (2). This chapter summarizes the diagnosis and management of allergic rhinitis in the aged individual, and discusses these in the context of the patient with significant other medical problems. We assume the reader has a basic knowledge of the evaluation and management of allergic rhinitis, and the basic diseases, pathophysiologies, and constitutional milieu associated with the geriatric age group (3–8). The proportion of people over age 65 in the United States is increasing faster than the general population (Table 1) (9). This is occurring despite continued socioeconomic incentives for smaller families! With the dramatic rise in the percentage of the population over 65, and over 85, there is a changing attitude toward health in the aged person (9–11). Allergic rhinitis is a common disease, with nearly 50 million Americans afflicted (12). Of allergic asthmatics, 90% have concomitant allergic rhinitis. Between 35% and 70% of people presenting to a general otolaryngology practice have allergic factors in their symptom complex (13,14). Physicians of tomorrow will have a large, expanding population of elderly patients with a continuum of physiologic changes and a complexity of medical problems impacting the management of common problems such as allergic rhinitis.

BACKGROUND To better understand the approach to managing allergic rhinitis in the geriatric patient, we will look briefly at general changes that occur in the body with aging. 213

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Table 1 Age Trends and Projections for the U.S. Population Census year 1900 1950 2000 2050 (projected)

U.S. population (million)

Percent over age 56

76 152.3 276.1 403.7 (projected)

4.1 8.2 12.6 20.3 (projected)

Recently, interest and research in immunogerontology has accelerated as the role of the immunoneuroendocrine system in the aging process and its effect on survival became apparent. Immunosenescence involves a decline, deregulation, and remodeling of the immune system as it interacts with other systems. This complex field is challenged to determine whether abnormal findings are related to disease in aged unhealthy patients, ‘‘normal’’ changes in aging patients, or immune effects of exogenous factors such as medications or the state of physical conditioning (15). Two approaches have helped sort out some of these conundrums: studying centenarians and the SENIEUR protocol. Anyone living to 100 years of age without major illness was assumed to be prototypic of a healthy, aged person (16). The immune systems of these centenarians appear to be more preserved, less deregulated, and at times, of different remodeling than aging people afflicted by cancer, infection, autoimmunity, and other diseases associated with old age (9). The SENIEUR protocol, on the other hand, uses rigid laboratory and clinical criteria to define a pool of healthy aged people for the purposes of study (16–18). Here are a few of the highlights of immunosenescence studies. The thymus gland involutes with age, impacting both innate and adaptive immunity (19–22). Thymic involution accelerates after puberty, giving a 60-year-old less than 5% of the thymic mass of a newborn. Capacity to respond to new antigens depends on na€
ve T cells (quiescent lymphocytes) found in the thymus. These cells are activated as antigens are processed, as part of the body’s attempt at antigen elimination. These cells become memory T cells, and are quiescent until re-exposure to that specific antigen. Young people have a high na€
ve-to-memory T-cell ratio, and aged individuals reverse the ratio, as would be expected, with time and continued exposure to numerous antigens. As the thymus involutes, the production of na€
ve T cells is reduced and the capability of new antigen response declines (16). With thymic involution, we would expect helper T-cell function to decline, but B-cell behavior changes too. These changes include modification in B-cell varietal expression, primary and memory B-cell production, and fluctuations in the ratio of B cells found in the marrow versus the spleen (15,23–26). With aging, the circulating population of B cells does not change, but production of antigen-specific antibody decreases and autoantibody production increases. Susceptibility to infections such as bronchitis, pneumonia, gastroenteritis, influenza (poor vaccination response), and autoimmune diseases increases (15,27–30). Other B-cell-related diseases such as B cell leukemia/lymphoma, monoclonal gammopathy/myeloma, etc. become more common (15,31). In healthy, elderly individuals, immunoglobulin G (IgG) and IgA levels actually increased, with all of the IgG subclasses increasing except IgG4 (15,32). Since IgG4 is regarded as a blocking antibody competing with IgE in the allergic response, a decrease in IgG4 may worsen allergy symptoms. IgE production tends to decrease in the older patient, which can lead to less intense allergy symptoms (33). Atopy may play an

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important role in complex respiratory complaints of geriatric patients. The paucity of knowledge in this area points to a need for research in geriatric allegroimmunology. Changes in specific cellular immune response and T-cell response affect much of immunosenescence. In addition to thymic involution and decreasing na€
ve-to-memory T-cell ratio, T-cell receptor stimulation and response to receptor stimulation are decreased (31,34). Changes in CD4þ T cells and the subsequent alterations in interleukin (IL)-2 production favor a proliferation decline. Some studies suggest decreased IL-10 and increased IL-4 and IL-6 production. Changes in interferon (IFN)-g have been variable. Cytotoxicity and delayed hypersensitivity do decline with age (27). Innate immunity integrates with antigen-responsive T- and B-cell function (35). This includes phagocytosis, some cytotoxicity, production and release of cytoactive molecules such as adhesion molecules and cytokines, chemotaxis, and intercellular responses. Natural killer T cells usually increase with advancing age. Efficient natural killer activity is found in healthy geriatric individuals, while those with decreased activity often have increased morbidity and health problems. The general decrease in cytokine production with advanced age impacts the T-helper-1 (Th1)/Th2 helper T-cell balance. IgE production and thus allergic symptoms depend on the Th2 lineage. The cell-related cytokine profiles shift with age. Th2related immunity is prominent in infants, shifting to a Th1 profile in adulthood, and trending back to Th2 with advancing age. These immune changes parallel a common clinical presentation, of childhood allergy symptoms that were ‘‘outgrown,’’ and then tend to recur with advancing age. Age-related physiologic changes can worsen the impact of allergy symptoms. The geriatric population tends to be relatively dehydrated, causing more viscous mucus (36). Mucous-secreting glands decline and mucociliary transport is altered, so mucus is not transported efficiently. Atrophic changes are compounded by reduced nasal blood flow, and the nose becomes drier. The patient experiences more nasal congestion, stuffiness, and a perception of increased postnasal drainage, and becomes more susceptible to infection (37–46). Age-related structural nasal changes add to nasal obstruction and increased airway resistance. The nasal tip drops, and the columella retracts backward and upward (47). Thinning of upper and lower nasal cartilages paired with muscle atrophy promote nasal alar incompetence, and nasal obstruction.

PATIENT PRESENTATION AND HISTORY Virtually every patient complaint relating to the nose or paranasal sinuses should prompt the taking of an allergic and environmental history (3,6). A well thought out patient questionnaire can be time efficient and promote thoroughness. Medication: About 400 brand-name drugs have had rhinitis as a listed side effect (48,49). These drugs include some antihypertensives, aspirin and nonsteroidal anti-inflammatory agents, oral and parenteral contraceptives, conjugated estrogens, beta-blocking agents, some psychotropic drugs, cocaine, and legal topical vasoconstrictors commonly associated with rhinitis medicamentosa. Review of the patient’s medication list will identify the possibilities. A suspect drug must be considered in consultation with the prescribing physician, weighing the importance of that medication in treating illness, and what options exist for alternate medications. Head and Neck: Recurrent infections (rhinosinusitis, conjunctivitis, otitis, pharyngitis, laryngitis) can be exacerbated by inhalant allergies. IgE production

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increases in the allergic patient during viral infection, correlating with the clinical observation that the allergic patient often has a more prolonged illness and an increased risk of secondary bacterial infection (50). Dry eye syndrome can mimic or coexist with allergic conjunctivitis. A history of childhood allergic symptoms with an improvement in adulthood and a recrudescence at an older age is not uncommon. Cardiovascular: Increased nasal congestion can occur with congestive heart failure. A history of hypertension, arrhythmia, valvular disease, cardiac surgery, peripheral vascular disease, etc. may affect the decision to use alpha-adrenergic drugs. Beta-blocker medications are a relative contraindication to allergy skin testing or immunotherapy, as they alter the effect of epinephrine if required to treat a systemic reaction or anaphylaxis. Angiotensin-converting enzyme (ACE) inhibitors can predispose to angioedema in allergy patients (51,52). Respiratory: A patient with asthma (including cough-variant asthma) should be evaluated for allergies. A patient with allergic rhinitis has a 30% chance of developing asthma during a lifetime, and 90% of asthmatics have rhinitis. Asthma may have its onset in the geriatric age group (53,54). Recurrent bronchitis, bronchiectasis, chronic obstructive pulmonary disease (COPD) (including emphysema), and chronic cough may all have asthmatic/allergic components. Increased nasal airway resistance can contribute to obstructive sleep apnea and COPD (55). Gastrointestinal: Laryngopharyngeal reflux and gastroesophageal reflux disease (GERD) occur frequently and can exacerbate asthma and voice or throat complaints. Food sensitivity is sometimes allergic in origin, so a history of colic in infancy, childhood gastrointestinal (GI) symptoms, or suspected food allergy should prompt further investigation. Some food allergens cross-react with other food or inhalant allergens, as seen in the ‘‘oral allergy syndrome.’’ Nasal inflammation can occur with inflammatory bowel disease. Genitourinary: Bladder outlet obstructive symptoms can influence the choice of medication used for allergy treatment. Childhood enuresis can suggest allergy (56). Musculoskeletal: Rhinitis is increased with collagen vascular diseases such as rheumatoid arthritis and lupus erythematosus. It is similarly increased in granulomatous diseases such as sarcoidosis and Wegener’s granulomatosis (57). Integumentary: A history of eczema, hives, or angioedema (even if remote) suggests an allergic origin to a patient’s rhinitis. Eczema or atopic dermatitis is associated with food allergy in children (30–80%) and inhalant allergies in adults. Atopic dermatitis is complex in character with other exacerbating factors including (but not exclusively) skin moisture/dryness, irritant exposure, and bacterial/fungal colonization (Staphylococcus aureus, Malassezia sp.) with possible super antigen effects (52,58). Endocrine: Endocrine disorders associated with rhinitis include pregnancy, some birth control medications, diabetes, and thyroid dysfunction. Delivery or correction of the underlying problem may not lead to complete symptom resolution. Allergy evaluation and treatment can provide symptom improvement. Obesity can also contribute to nasal congestion. Psychologic: Poor control of psychiatric problems such as anxiety and depression can magnify the effect of rhinitis symptoms on quality of life. Psychiatric medications may have nasal side effects (rhinitis, mucosal dryness). Tricyclic antidepressants and monoamine oxidase inhibitors are contraindications to allergy skin testing and immunotherapy. Hematologic: While frequent rhinosinusitis may be suggestive of allergic factors, immunodeficiency and hematologic conditions need consideration.

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Geriatric patients often have multiple medical problems, and take multiple medications. When undertaking evaluation and treatment of allergies, coordination of care with the patient’s primary care physicians or other specialists will ensure the best possible treatment outcome. DIAGNOSIS It is often appropriate to begin an empiric trial of allergy pharmacotherapy based on the patient presentation. Suboptimal response of symptoms to initial pharmacotherapy is the most common reason for allergy testing. The information gained from allergy testing is used to design environmental changes to minimize allergen exposure, and as a basis for immunotherapy. There are some age-related considerations in considering in vivo or in vitro allergy testing. Skin test reactivity declines with advancing age (39,59–64). This occurs despite a favored Th2 function, and is related to declining total IgE levels, tissue remodeling, and atrophic changes. Intradermal skin testing is more sensitive than epidermal (prick or scratch) testing, and may therefore be more appropriate in elderly patients. Systemic and intranasal antihistamine medication must be stopped three to five days prior to skin testing. In general, patients on beta-blocker medications, tricyclic antidepressants, or monoamine oxidase inhibitors are not skin tested. Several classes of medication may interfere with the accuracy of allergy skin testing. These include antihistamines (both older and newer nonsedating types), some psychotropic drugs, especially the tricyclic antidepressants, nontopical corticosteroids in medium to higher doses, H2 blocking antihistamines, and some leukotriene modifying agents. Beta-blocking agents do not affect the accuracy of skin testing, but can impede epinephrine action if the patient requires treatment of a systemic or anaphylactic reaction due to skin testing. Topical beta-blockers such as ophthalmic preparations for glaucoma carry the same risk as oral beta-blockers (56). If these medications cannot be withdrawn for testing, in vitro testing, which is not affected by the patient’s drug profile, is recommended. In vitro antigen-specific IgE testing is sometimes a better choice for the geriatric patient. Many elders are on medications interfering with the accuracy or safety of allergy skin testing (as above, systemic dose steroids, antihistamines). Common comorbidities can impact the safety of allergy skin testing, such as the patient with rhinitis and asthma-factored COPD and little pulmonary reserve who could develop asthma during testing, or the brittle cardiac patient with suspected allergy and a relative contraindication to epinephrine use should an adverse reaction to testing develop. Other patient situations favoring in vitro testing include abnormal skin reactivity (dermatographism or urticaria), needle phobia, or a history of anaphylaxis. MANAGEMENT Elimination or avoidance of offending allergens is the cornerstone of allergy management. This can eliminate symptoms in a patient with limited sensitivities, and can reduce the allergic load in more complex patients. Environmental control may be difficult for some geriatric patients due to finances, an unfriendly domiciliary situation, or physical difficulties interfering with performing the required cleaning tasks. Living in a communal environment such as a nursing or retirement home may result in a limited say in controlling the surroundings.

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For the dust mite-allergic patient, the key is making the bedroom and other frequently used areas as dust-free as possible. Strategies include allergen-proof mattress and pillow covers, weekly washing of bedclothes in hot water, meticulous dusting, and elimination of nonessential soft furnishings such as draperies and carpets. Laundry additives, carpet treatments, high-efficiency vacuum cleaners, and HEPA filters can also be helpful in minimizing dust exposure. Mold-avoidance strategies include thorough frequent cleaning of all humid areas of the home (bathrooms, laundry rooms), routine emptying of drip pans (under refrigerators, air conditioners), elimination of houseplants, and dehumidification. Gardening and lawn mowing also expose the patient to heavy mold loads, and should be avoided. Pet allergies can be controlled by giving away a pet or keeping the pet outdoors. When the pet in question is an important family member, however, such strategies will not work. Weekly washing and grooming of the pet and keeping the pet out of the bedroom will help with symptoms. Pollen-allergic patients can avoid exposure by spending as little time outdoors as possible during pollination season. Broadly speaking, this is spring for tree pollens, summer for grass pollens, and fall for weed pollens. Local weather, plantings, and microclimates, however, provide much variation in pollination seasons. Wearing a mask covering the nose and mouth can minimize inhaled pollen. When coming in from outdoors, performing nasal irrigations, showering, shampooing, and completely changing clothes also reduce pollen exposure. Pharmacotherapy for control of allergic symptoms has an annual ‘‘price tag’’ of several billion dollars in the United States (12,55,65–67). Successful drug choices for pharmacotherapy improve the symptoms, while not interfering with management of other medical problems, not causing serious side effects, and being affordable and easy to use. These choices become more complex in the context of the physical, psychological, and socioeconomic challenges of advancing age. The general classes of medications used in management of allergic rhinitis include antihistamines (oral and topical), alpha-adrenergic decongestants (oral and topical), leukotriene modifying agents, topical anticholinergic agents, mast cell stabilizers, corticosteroids (oral, topical, and parenteral), and mucolytic agents. Antihistamines improve symptoms of allergic mucosal inflammation and irritation. All antihistamines are equally efficacious. Side effects of first-generation antihistamines that may impact the elderly include mucosal dryness, increased mucus viscosity, altered mucociliary function, sedation and slowed reaction time, cardiotropic effects, hypertension, weight gain, hair loss, and bowel/bladder dysfunction. In the newer generation of antihistamines, these side effects are reduced or eliminated. Topical antihistamines also have some of these side effects, especially sedation and a local drying effect. Oral decongestants help shrink edematous mucosa, improving nasal obstruction. Side effects include numerous cardiotropic effects, including hypertension, arrhythmias, and angina. Bladder, prostate, and bowel dysfunction are common, as are sleep disturbances. Topical preparations, including both the sympathomimetics and the imidazoline derivatives, cause some of the same systemic side effects, as well as carry the risk for rhinitis medicamentosa with prolonged use. Corticosteroids have numerous side effects. Used as directed in recommended doses, intranasal steroids do not promote lasting hypothalamic–pituitary–adrenal axis changes, bone growth suppression, osteoporosis, or other worrisome effects (68,69). Airway dryness and epistaxis (especially in the anticoagulated or platelet-adhesive-treated

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patient) are not rare. Age-related physical and cognitive difficulties may render both use and compliance difficult, especially when using nasal sprays and eye drops. Some patients may be ‘‘steroid responders’’ from the standpoint of ophthalmic susceptibility to an increase in intraocular pressure, or cataracts, with the application of topical ophthalmic steroids, using on-label prescribed doses (70). Lastly, a miscellany of thoughts on management medications is in order. While water is the best mucolytic agent for the dehydrated patient, guaifenesin is the most commonly used. If guaifenesin is not used with adequate hydration, it has a potential drying side effect. Data support the nasal decongestant effect of the leukotriene modifying agent, montelukast, which is attractive in the patient at risk for the cardiotropic effects associated with the alpha-adrenergic decongestants (71,72). Anticholinergic topicals, while safe and relatively free of side effects, are of limited use in allergic rhinitis and are most useful for vasomotor or idiopathic rhinitis factors. Saline topicals may not only be used for moisturizing, but may also have a symptomatic, therapeutic use. Zealous overuse of saline sprays may actually promote dryness due to an evaporative effect on the nasal mucosa. Saline gels may help reduce this consequence. Drugs in development that may provide therapeutic alternatives for elderly patients include more potent intranasal steroids (indicated for younger and older patients), oral H1 plus H2 antihistamine combinations, oral antihistamines new to the U.S. market (tecastemizole, ebastine, mizolastine), more potent intranasal antihistamines, combination steroid plus antihistamine intranasal sprays, and leukotriene plus antihistamine combinations. Additional future options include pharmaceutical substances such as anti-IgE, which blend the lines between pharmacotherapy and immunotherapy.

IMMUNOTHERAPY As with younger patients, immunotherapy is usually reserved for the patient with multiple sensitivities who is not responding well to pharmacotherapy and avoidance strategies. Since the geriatric patient’s medical condition tends to be more complex, however, special attention is paid to risk and the patient’s potential reserve for recovering from an adverse reaction to therapy, should one occur. Benefits of immunotherapy include better symptom control, and diminished need for medications. Sublingual immunotherapy has been used with success outside the United States, and is now coming under research scrutiny within the United States. Issues of cost (for example, eliminating charges for allergy injections), safety, drug interference issues, and compliance make this modality attractive to patients and payors. Tablet-based immunotherapy has been used with some success in Europe. Consideration for this single antigen, sublingual tablet will need appropriate U.S. research to complete development and obtain Food and Drug Administration (FDA) approval before launching in the United States. Anti-grass tablets will possibly be the first of potentially several formulations to be produced. Anti-IgE therapy is now approved in the United States for the management of adult forms of moderate to severe IgE-mediated asthma (73). Omalizumab is the (rhu)mAb-E-25 antibody, which is a recombinant humanized monoclonal antibody to IgE-25 derived from the murine antibody MAE-1. Indications will be sought for the pediatric age group and then for allergic rhinitis (74). Studies for allergic rhinitis are making their way into the literature now (75–79).

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Other mediator antagonists and immune modulators are in the earlier stages of research. Soluble IL-4 receptor antagonists, anti-IL-4 monoclonal antibodies, and mutated IL-4 receptor antagonists are being studied. Humanized anti-IL-5 antibodies for use in asthma, IL-5 synthesis inhibitors, and murine anti-IL-5 antibodies are works in progress. Other considerations include anti-IL-13 monoclonal antibodies, soluble IL-13 receptors-Fc fusion proteins, eosinophil receptor CCR-3 antagonists, adhesion molecule inhibitors, chemokine [RANTES, Eotaxin, monocyte chemotactic and activating factor (MCAF), monocyte chemotactic protein-1 (MCP)-1] modulation, action against mast cell secretory granule enzymes (tryptase, chymase), recombinant IL-12 (Th1 function suppresses Th2/allergic function) preparations, the nebulization of IFN-g for topical asthma treatment, prostaglandin modifying agents, and more to come (80,81). There is also a future in other forms of immunotherapy. Local nasal immunotherapy and peptide-based immunotherapy are nearing the horizon. Oligonucleotides are immune boosting molecules, which are attached to antigens and then the patient is injected. Enzyme-potentiated desensitization has been in use, but will need more research and regulatory alignment before becoming an easily available tool (82). While all of these modalities are provocative to thought, and may help reduce the need for conventional pharmacotherapy, consideration as to how these forms of allergy treatment will affect other aspects of the patient’s medical make-up are critical, especially in the geriatric patient. FOOD ALLERGY Adverse reactions to foods are immunologically mediated (immediate IgE-mediated vs. delayed immune reactions). The most common immediate IgE-mediated reactions occur with peanuts and seafood. The diagnosis is usually made by history, and absolute avoidance is the only treatment. Patients with this condition should always carry self-injectable epinephrine (EpiPen1) for use in cases of inadvertent exposure. The remainder of adverse reactions to food is best diagnosed by a careful history, a food diary, and withdrawal of the suspect food for five days, followed by reintroduction. Any foods that cause symptoms are eliminated from the diet completely for at least four to six months. After that time, patients can often tolerate occasional meals containing that food. If the food is eaten too frequently, however, symptoms will recur. SUMMARY Allergic rhinitis is a common and costly disease. A careful history will suggest an allergic etiology for the patient’s symptom complex. Empiric pharmacotherapy often yields symptom resolution. When pharmacotherapy does not improve symptoms enough, allergy testing (skin or in vitro testing) can identify the specific allergen. Avoidance strategies can be planned, and, if needed, immunotherapy can be begun. ACKNOWLEDGMENT The author wishes to thank Ms. Karen Stoner, Chief Librarian of the Decatur Memorial Foundation Library for Ms. Stoner’s tireless efforts, and the American Academy of

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Otolaryngic Allergy Foundation library. Further thanks to Mr. George Smaistrla Jr., Clinic Administrator, and Ms. Julie Causey, transcriptionist, for their assistance. Lastly, the author’s warm thanks goes to Melinda for donating time, and to Nicholson with whom the author divides the present with Vanderbilt University.

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20 Rhinitis in the Aging Patient Margaret A. Chen and Terence M. Davidson Division of Otolaryngology—Head and Neck Surgery, VA San Diego Healthcare System, and Department of Surgery, UCSD Medical Center, San Diego, California, U.S.A.

INTRODUCTION Aging rhinitis, an atrophic rhinitis, is a common ailment in the elderly population and results from normal aging processes in the nose. Morbidity is significant, with patients complaining of throat tickle, thick postnasal drip, and chronic cough. Many do not seek medical treatment for this process, and those who do are frequently misdiagnosed or inappropriately managed. Fortunately, there is a simple and effective treatment. While medications and surgery have little utility in this disease, nasal irrigation with pulsatile, hypertonic nasal saline irrigation has been shown to be very effective in relieving symptoms and should be the standard of treatment. This chapter will discuss the pathophysiology, presentation, and treatment of aging rhinitis, including a comprehensive review of nasal irrigation.

OVERVIEW OF NORMAL NASAL PHYSIOLOGY Nasal mucosa has two populations of cells responsible for secretory function. These are the nasal mucosal goblet cells, which produce a thick tenacious mucus, and submucosal serous glandular cells, which produce a thin watery mucus. The mucus itself is divided into two phases. The outer mucoid gel phase is viscous and traps particulate matter. The inner watery sol phase is thinner and less viscous. The mucus blanket floats on the watery sol layer. Ciliary beating in the sol layer propels the mucus blanket from the sinonasal cavity to the nasopharynx. Healthy nasal mucosa produces approximately 1 L of mucus each day, which is normally swallowed. Mucus properties are critical to nasal health. Changes in mucus viscosity disrupt the normal cycle of flow and cause troublesome nasal symptoms (1,2). Turbinate vasculature is primarily responsible for warming and humidifying inspired air. This system is regulated by autonomic control and is highly sensitive to changes in temperature or circulating hormones. Changes in vascular health have severe consequences on normal mucosal function.

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Figure 1 Pathophysiology of aging rhinitis.

PATHOPHYSIOLOGY OF AGING RHINITIS Clinical Changes Several factors contribute to the symptomatology of the aging nose (Fig. 1). The mucus becomes thick and tenacious secondary to generalized decreased body water content and local serous glandular atrophy (3). Change in the gel and sol layers impairs mucociliary function, resulting in mucosal stasis. The thick mucus is suspended in the nasopharynx and causes symptoms of throat tickle, snorting, postnasal drip, and cough (4,5). An excellent clinical study by Edelstein involving 111 healthy subjects ages 21–94 showed that postnasal drip is 35% more prevalent with every 10 years of age (6). Other nasal complaints that occurred significantly more frequently with age were nasal drainage, sneezing, and coughing. Autonomic dysfunction and atherosclerosis in aged individuals alter the vascular tone of the nasal turbinates. Decreased humidification of inspired air results in bothersome crusting. Thinner mucosa and fragility of vessels result in more frequent epistaxis. Ironically, turbinate atrophy can cause ‘‘empty nose-like syndrome,’’ i.e., atrophic rhinitis, where nasal obstruction is falsely perceived secondary to disrupted perception of normal airflow. Histopathologic Changes In 1944, Hollender (7) published an extensive histopathologic comparison of nasal mucosa in 23 cadavers aged 50–90 years. Hollender was unable to find a correlation between epithelial morphology and age. However, older subjects demonstrated a

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relative decrease of lymphatic tissue in the subepithelial layer with abundant fibrosis. In 1992, a follow-up scanning electron microscopic study of aging human nasal epithelium demonstrated that the number of ciliated cells on the inferior turbinate did not decrease with age (8). Rather, the authors postulated that drying and crusting in the aging nose are likely due to changes in the viscoelastic properties of mucus. Structural Changes The structure of the nose significantly changes with aging. Degeneration of collagen and elastin in the skin with atrophy of subcutaneous tissues and facial musculature leads to drooping of the nose (9). Resorption of premaxillary fat and changes in dentition contribute significantly to this process. The result is a downward rotation of the lobule and retraction of the columella. The most important structural change occurs at the junction of the upper and lower lateral cartilages. Weakening of fibrous connective tissue at this location causes a significant loss of nasal tip support. Aging cartilage undergoes disorganization of its collagen fibril architecture and proteoglycan aggregates (10,11). Ultimately, degeneration of key support structures such as the septal, upper, and lower lateral cartilages and their fibrous connections results in collapse of the nasal valve. Consequent nasal obstruction is greatly exacerbated by any pre-existing septal deviation.

Mucociliary Changes Mucociliary dysfunction in aging is more likely due to changes in mucus properties than to cellular derangement. Healthy elderly subjects were shown to have significantly impaired mucociliary transport relative to younger subjects, using the saccharin clearance test. Nevertheless, it should be noted that 70% of the elderly subjects had normal saccharin clearance times (12). While Edelstein’s clinical study of 111 healthy subjects did not show a significant change in ciliary beat frequency with age, the standard deviation of the intrasubject beat frequency did vary significantly with age (6). Immunologic Changes Dysregulation of the immune system, or immune senescence, has been studied in rat nasal mucosa (13). Aging mucosa showed an alteration in the synthesis and release of cytokines with alterations at receptor sites, resulting in loss of control of inflammatory processes. These findings are interesting in light of the inflammatory mechanisms associated with many conditions of the aging nose. Together, changes associated with aging result in disruption of mucus production and nasal airflow, causing symptoms of nasal obstruction and nasal dryness. Mouth breathing can result, which requires more physiological work than nasal breathing.

CLINICAL ASSESSMENT Presentation Commonly, patients who seek medical attention for aging rhinitis will complain of ‘‘allergies’’ or ‘‘sinus trouble.’’ Direct questioning will reveal the major symptoms

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to be thick postnasal drainage with constant throat clearing, snorting, and cough. The patient will complain of nasal obstruction, especially when lying down, and may note nasal crusting which is particularly bothersome on first awakening.

Medical History A thorough history should cover nasal and sinus surgery, nasal trauma, allergies, and concurrent medical illnesses. The patient will often have seen multiple medical professionals for this problem and all treatments, typically including nasal steroids and antihistamines, should be elicited. The patient’s complete list of medications must be reviewed, particularly for medications that may exacerbate nasal dryness, such as antihistamines, antidepressives, diuretics, and antihypertensives (14).

Physical Examination A thorough head and neck exam should be performed. On anterior rhinoscopy, it is common to see dry, irritated mucosa in a widely patent nasal passage, sometimes with crusting. Nasal airway obstruction should be evaluated. The nose should be examined endoscopically for infection, anatomic abnormalities, atrophic changes, and thick, viscous secretions. If a flexible endoscope is used, the postnasal secretions can often be seen stranding down the pharynx, often suspended over and into the larynx. If available, acoustic rhinometry will confirm that the nasal passages are widely patent.

Differential Diagnosis Other chronic sinonasal disorders may manifest similarly, such as chronic sinusitis, allergic rhinitis, and other atrophic rhinitides. Chronic inflammatory diseases (sarcoid, lupus, Wegener’s) and the irradiated nose may also demonstrate similar nasal physiology. These conditions should be considered during history and physical examination.

MANAGEMENT General Principles The primary goal of treatment for aging rhinitis is to wash out the thick nasal secretions. The most effective way to do this is with pulsatile, hypertonic, nasal saline irrigation. Other irrigation methods and adjunct treatments have also been widely used. While many controversies surround the issue of nasal irrigation, including saline tonicity, delivery of systems, and additives, the most important factor in symptom relief is frequent and regular irrigation. It must be emphasized to patients that control, not cure, is the goal of management for their disorder. The following section of the chapter will review literature evidence for the various aging rhinitis management strategies. Aging rhinitis is commonly misdiagnosed as allergic rhinitis, and patients are commonly prescribed antihistamines. However, the basic problem in aging rhinitis is the drying and thickening of nasal secretions. Antihistamines, which decrease mucus secretion in the nose, are contraindicated and will exacerbate aging rhinitis.

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Moreover, many antihistamines, particularly first-generation medications, have anticholinergic side effects that can cause significant morbidity in the elderly. Unless true allergic symptoms are present, antihistamines should be avoided in these patients. Nasal steroids are of no value in the treatment of aging rhinitis. Guaifenesin at a moderate dose (under 2400 mg/day) has mucolytic and expectorant properties, and is commonly prescribed for upper respiratory disorders. However, a double-blind placebo-controlled crossover trial of guaifenesin in 10 subjects showed no measurable effect on mucociliary clearance (15). In our experience, we have not found this medication to be particularly useful; moreover, it is associated with a high rate of gastrointestinal side effects.

Nasal Saline Irrigation The mainstay of treatment for aging rhinitis is nasal saline irrigation. Irrigation is used for many sinonasal disorders, and is thought to improve symptoms by several mechanisms. The primary mechanism is a mechanical clearance of inspissated secretions and crusts. Clearance of thick mucus improves mucociliary function, thereby improving mucosal health and function. Irrigation moisturizes the dry nasal cavity, while decreasing mucosal edema and clearing accumulated inflammatory mediators. There are no reported serious adverse effects to irrigation. Abundant subjective and objective evidence exists that nasal saline irrigation is efficacious in the treatment of sinonasal disease. Papsin and McTavish (16) have written an excellent literature review summarizing that nasal irrigation is simple, effective, and safe. Moreover, prescribing saline irrigation can help conserve medical resources. Saline irrigation is associated with lower utilization of medications and office visits. Because patients are often prescribed antibiotics by primary care providers for sinonasal complaints, decreasing medication use in this population can help lower the prevalence of antibiotic resistance. A clinical study of 93 chronic rhinitis patients showed significant improvement in patient symptoms with the use of metered-dose saline. Subjects also noted subjective improved efficacy of their pre-existing rhinitis medications, such as nasal steroids (17). A prospective, randomized, controlled, double-blinded trial of 40 sinusitis patients demonstrated that irrigation with saline solution resulted in significant improvement of subjective complaints, endoscopic appearance, and radiographic appearance (18). In a randomized controlled trial evaluating the value of hypertonic saline irrigation in 76 sinusitis patients, subjects were randomized to irrigation with 2% hypertonic saline or no irrigation (19). Both groups continued their usual medical regimen for sinusitis. Subjects receiving irrigation had significant improvement of symptoms over the no-irrigation group by quality of life scores from two questionnaires. Of note, all patients who complied with a debriefing questionnaire indicated that they would recommend irrigation to friends and family, and 95% indicated that they would continue to use nasal irrigation. A prospective controlled clinical study of 211 nasal dysfunction patients demonstrated that irrigation with pulsatile hypertonic saline significantly improved both severity and duration of patient-rated nasal symptoms (e.g., nasal congestion, postnasal drip, allergies, and nasal discharge) (20). Quality of well-being, assessed by the QWB questionnaire, also significantly improved. Moreover, compliance with irrigation was 92% after six weeks.

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Irrigation Delivery Methods of irrigant delivery may be as simple as a cupped hand or as elaborate as a top-of-the-line irrigating device. Other popular methods are bulb syringe, spray pump, nebulizer, and irrigation, or Neti, pot. Passive instillation differs from pressurized, or stream, delivery. Stream delivery may further be differentiated into laminar or pulsatile flow. The literature is rife with comparisons of efficacy among these methods. A 99 m-technetium radio-labeled saline study demonstrated that a nebulizer is superior to a spray pump in distributing aerosol to the nasal cavity (21). A similar study using dye solution and endoscopy compared delivery among aerosolized spray, atomizer, nebulizer, and bulb syringe (22). While there was no demonstrable difference between aerosol and atomizer distribution, bulb syringe appeared to be statistically superior to other delivery methods in multiple sinonasal sites. Other evidence further supports the efficacy of stream delivery. A randomized study of nasal irrigation in a post-rhinologic surgery population showed that stream delivery was significantly more effective than drops in improving both symptoms and endoscopic appearance (23). In a randomized controlled study, stream delivery by bulb syringe and by irrigation pot were equally effective in decreasing symptoms and medication usage among 150 chronic sinusitis patients (24). Stream delivery of irrigant is most effective when delivered in a pulsatile as opposed to a laminar fashion. A study from the orthopedic literature demonstrated that pulsatile irrigation was 100 times more effective than bulb syringe in removing slime-producing Staphylococcus from implant surfaces, regardless of what irrigating solution was used (25). A radiologic study using contrast-labeled irrigation with computed tomography (CT) scan compared negative and positive pressure irrigation to nebulization (26). While both positive and negative pressure distributed contrast effectively, distribution was more even with positive pressure. Nebulization, however, distributed contrast poorly. In 1974, Grossan (27) published a report of a new nasal irrigator tip to provide pulsatile irrigation from a WaterpikTM dental device. This new method made pulsatile nasal saline irrigation much more accessible to patients because it was inexpensive, readily available, and simple to use. To this day, it remains a very widely used and efficacious therapy. The potential disadvantage of pulsatile irrigation involves trapping of fluid within the sinuses or the middle ear. Theoretically, this may result in serous otitis media or sinus pressure. If this occurs, the patient may need to change their method of irrigation to a laminar system, such as a Neti pot or syringe delivery system. Irrigant Solutions Saline Tonicity A great deal of conflicting literature and opinion exists as to whether hypertonic saline is more efficacious in nasal irrigation than isotonic normal (0.9%) saline. Hypertonic saline has been studied in various concentrations and is believed to have several mechanisms of efficacy. First, salt has a topical antibacterial effect that is generally concentration-dependent. Second, the high ionic concentration disrupts ionic bonds in mucus, thus decreasing its viscosity. Third, diffusion of osmolar gradients may reduce mucosal edema. Fourth, hypertonic saline increases the mucinous response of nasal mucosa. Finally, high concentrations of salt stimulate release

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of intracellular calcium, possibly facilitating the use of adenosine triphosphate (ATP) at the ciliary axoneme. Increased ciliary beating improves mucociliary clearance. The antimicrobial effect of hypertonic saline has been described in the surgical literature. One study described the use of different concentrations of saline to dress ulcers (28). Only ulcers dressed with hypertonic saline were culture-negative. Inoculation of Staphylococcus into different concentrations of saline showed that growth was not inhibited by normal saline, but was completely inhibited by 8.8% (3 Osm/L) saline. However, patients whose ulcers were dressed with 8.8% saline complained of itching at the site. The authors concluded that a moderate saline concentration of 4.4% (1.5 Osm/L) was the best compromise for antimicrobial effect and patient tolerance. In a prospective crossover study comparing normal saline to hypertonic saline, 21 healthy subjects were randomized to a single irrigation with either normal or hypertonic (3%) saline (29). Subjects then underwent irrigation with the other solution the next day. Mucociliary transport, using the saccharin clearance test, was assessed at baseline and after each irrigation. There was a significant improvement in mucociliary transport only after irrigation with hypertonic saline. Although this study was conducted on healthy subjects, the authors predicted an even greater effect in symptomatic patients, where viscoelastic properties of mucus are likely altered. A randomized, double-blind study of 30 children with chronic sinusitis compared normal and hypertonic (3.5%) saline nasal irrigation (30). Outcome was measured by three scores: cough score, nasal secretion/postnasal drip score, and radiologic Water’s view score. Significant improvement in all three scores was seen with hypertonic saline, but only the nasal secretion/postnasal drip score was improved with normal saline. The effect of hypertonic saline mucin secretion was studied in 16 healthy subjects (31). Subjects underwent hypertonic saline lavage, followed by histamine or methacholine challenge. Hypertonic saline alone was able to evoke global mucinous secretion, and also potentiated mucinous secretion after histamine or methacholine challenge. One randomized, controlled, double-blind trial concluded that hypertonic nasal saline is not efficacious (32). One hundred forty-nine patients with colds or acute sinusitis were randomized to hypertonic or normal saline spray, or observation. Analysis of nasal symptom self-rating showed no difference in symptoms or duration of disease. Moreover, more than half of the patients indicated that they would not use hypertonic saline again because of burning discomfort on administration. These results are in contradiction to findings of patient tolerance in the majority of published studies. It is possible that these patients, suffering from self-limiting illnesses, were less likely to tolerate a new treatment for questionable gain. Our experience, as well as that evidenced in the bulk of the literature, indicates that patients with chronic disease overwhelmingly take to nasal irrigation when properly instructed, with excellent return on symptom resolution. Base Solution While most studies have examined the efficacy of hypertonic or normal saline in nasal irrigation, some studies suggest a re-examination of the base solution choice. An in vitro experimental study examined ciliary beat frequency in sinus mucosa in different solutions (33). Normal saline, hypertonic saline (7% and 14.4%), and Locke–Ringer’s, an isotonic physiologic solution, were compared. While Locke– Ringer’s solution had no inhibitory effect on ciliary beat frequency, normal and hypertonic saline had significant inhibitory effects proportional to salt

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concentration. In fact, 7% hypertonic saline caused a reversible ciliostasis, while 14.4% hypertonic saline caused irreversible ciliostasis and even cytotoxicity. It is important to note that these experiments were conducted in vitro and not in vivo. Nevertheless, the authors raise the question of whether nasal irrigation might be even more clinically effective if solutions without known ciliostatic effects, e.g., Locke– Ringer’s, are used instead of saline. An in vivo study of postoperative septoplasty patients compared mucociliary transport in patients irrigating with lactated Ringer’s versus normal saline (34). Saccharin clearance was improved after irrigating with lactated Ringer’s over normal saline. Because lactated Ringer’s has the advantage of being more physiological in composition than normal saline, it may have less inhibitory effect on mucociliary function. Moreover, the solution, like saline, is inexpensive. However, given the composition of this solution [3.1 g sodium lactate, 6.0 g sodium chloride (NaCl), 0.3 g potassium chloride (KCl), and 0.2 g CaCl2
2H2O], this solution would be difficult to make at home and would likely have to be purchased for long-term use. Water Source The use of tap water in homemade irrigation solution is important. Tap water commonly contains disinfectants such as chlorine, ozone, chlorine dioxide, and chloramines. Disinfection byproducts such as chloroform and trichloroacetic acid are also present. While some of these disinfectants and by-products are harmful in large doses, the amounts found in tap water are regulated closely by the Environmental Protection Agency to be at low levels such that the benefits of disinfection outweigh other risks (35). Chlorine is a well-known broad-spectrum disinfectant that is a welcome addition to the nasal irrigation regimen. We recommend the use of tap water over bottled water for solution-making at home. Of note, the hardness of the water does not have a deleterious effect on its antibacterial action. Additives and Antibiotics Bicarbonate The major indication for adding bicarbonate to irrigant solution is to ameliorate the burning sensation of hypertonic saline. If the patient finds irrigation uncomfortable for this reason, instructions should be given to add one-half teaspoon of baking soda to 500 cubic centimeters (cc) of solution. We believe that bacteria are more likely to thrive in an alkaline, rather than acidic, environment, and do not encourage the general use of bicarbonate in irrigant solutions. It should also be noted that chlorine is a more effective disinfectant at lower pH. Nevertheless, patient compliance with the nasal irrigation regimen is of the utmost importance. Moreover, evidence is contradictory on this topic. Traditional teaching is that an alkaline environment causes mucus to be in a sol, rather than gel, state. However, a study of mucociliary clearance after nasal douching demonstrated no difference between unbuffered solution and solution buffered to pH 8 (36). Antibiotics and Antifungals Antimicrobial medications can be added to irrigant solutions for topical delivery to the nasal cavity and sinuses. Tobramycin irrigation (20 mg in 50 cc saline once daily) is a valuable adjunct in the treatment of cystic fibrosis-related sinusitis (37).

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Table 1 Tonicity of Nasal Irrigant Solutions Normal saline (500 cc)

One teaspoon NaCl in 500 cc water

One heaping teaspoon NaCl in 500 cc water

0.9% 4.5 0.077 0.154

1.6% 8.0 0.137 0.274

2.3% 11.5 0.197 0.394

% NaCl (g/100 mL) Weight Nacl (g) Moles NaCl Molarity (mol/L)

It is assumed that table salt is 100% NaCl (most table salt is reported to be > 99% NaCl and < 1% calcium silicate. One teaspoon is 5 cc. MW NaCl ¼ 58.44 g/mol. Abbreviation: MW, molecular weight.

Tobramycin has also been successfully used in the management of HIV sinusitis, difficult-to-manage chronic sinusitis, and atrophic rhinitis, including aging rhinitis (38,39). The use of topical gentamicin for purulent atrophic rhinitis has also been described (40). The addition of antifungal agents for allergic fungal sinusitis is a relatively new advance. Nasal irrigation with amphotericin B suspension has been studied in 74 patients with nasal polyposis (41). After four weeks of twice-daily irrigation, nasal polyps disappeared in 39% of subjects. Efficacy was significantly higher in earlierstage disease. Of note, there were no controls in this study, and the risk of systemic toxicity with use of topical amphotericin B was not addressed. The use of pulsatile and nebulized amphotericin B for the treatment of invasive fungal sinusitis has also been described (42).

Directions for Patients A simple 1.6% hypertonic saline solution can be made by patients at home (Table 1). If burning is uncomfortable, one-half teaspoon of baking soda may be added. Patients should be instructed as follows: 1. Mix one level teaspoon of table salt in 500 cc (approximately two cups) of warm tap water (approximately body temperature). 2. Fill the nasal irrigator basin with the salt water. 3. Turn the unit on at the lowest setting. 4. Lean over the sink and place the irrigator up to the nose. Let the water run into the nose. It will run out the opposite side of the nose or the mouth. 5. Tilt and twist the irrigator side to side and up and down to direct the water flow into all portions of the nasal cavity until it feels clean on that side. 6. Repeat the procedure with the opposite side. 7. This can be repeated up to three times a day. The water pressure can be increased as the patient becomes accustomed to the sensation of nasal irrigation. Multiple excellent irrigation devices are commercially available (Fig. 2). Patients unable to tolerate pulsatile irrigation may still benefit from a laminar system using Neti pot or syringe delivery.

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Figure 2 Nasal irrigation systems. (Continued on next page)

Other Treatments Surgery Surgery does not have a significant role in the management of aging rhinitis. In fact, overzealous mucosal resection and manipulation during surgery can contribute to tissue atrophy and accelerate the symptoms of aging rhinitis. The sole indication for surgical therapy is in the case of a severe septal deviation causing nasal obstruction and inability to irrigate the nose effectively. Septoplasty or septorhinoplasty may then be indicated; however, resection of tissue must be conservative.

Alternative Treatments Several other therapies should be discussed for completeness. One alternative treatment is based on an ancient yogic treatment for rhinitis and involves the cleaning and rubbing of the nasal passages with a rubber catheter (43). This process likely works by eliciting a strong response of rhinorrhea, as well as the mechanical clearance of crusts and secretions. A study of reflexology massage on established sinus contact points on the fingertips and toes showed reflexology to be equally efficacious to nasal saline irrigation by bulb syringe or irrigation pot (24).

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Figure 2 (Continued)

Lubricants Little work has been done on the value of lubricating agents in the treatment of aging rhinitis. One randomized crossover study of sesame oil treatment in 79 subjects with nasal dryness demonstrated significant improvement in patient-rated nasal mucosa dryness, stuffiness, and crusting (44). Our experience is that, in general, lubricating agents are not terribly useful. While these may serve as a protective cover over the nasal mucosa, they do nothing to rid the nose of the tenacious mucus secretions responsible for the majority of patient symptoms. At best, they are a useful adjunct to nasal saline irrigation. Our favorite nasal lubricant, developed at the Massachusetts Eye and Ear Infirmary and brought to California by Dr. Jeffrey Harris, is a suspension of menthol, eucalyptus, and camphor in a mineral oil base.

CONCLUSION Aging rhinitis is a common disorder in the elderly and results from natural aging processes in the nose. The pathophysiology is based on the thickening and drying of nasal secretions, leading to a perceived increase in mucus and symptoms in the nose and pharynx. It is important not to misdiagnose this ailment as allergic

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rhinitis, since antihistamine medications commonly contribute to nasal dryness. The disease is chronic, and treatment is based on control rather than cure. The mainstay of treatment is nasal irrigation with pulsatile, hypertonic, saline tap water. While many irrigation methods have been described and debated in the literature, patient compliance with regular irrigation is the key to successful management of symptoms.

REFERENCES 1. Bassichis BA, Marple BF. Dry mouth and nose in the older patient: what every PCP should know. Geriatrics 2002; 57(10):22–35. 2. Jordan JA, Mabry RL. Geriatric rhinitis: what it is, and how to treat it. Geriatrics 1998; 53(6):76–81. 3. Janzen VD. Rhinological disorders in the elderly. J Otolaryngol 1986; 15(4):228–230. 4. Davidson TM. Handbook of nasal disease. (Internet. http://www.surgery.ucsd.edu/ent/ DAVIDSON/NASHAND/nasal.htm.) 5. Davidson TM. Otolaryngic gerontology (issues of aging). (Internet. http://www.surgery. ucsd.edu/ent/DAVIDSON/AGING/Aging.htm.) 6. Edelstein DR. Aging of the normal nose in adults. Laryngoscope 1996; 106(9 Pt 2):1–25. 7. Hollender AR. Histopathology of the nasal mucosa of older persons. Arch Otolaryngol 1944; 40:92–100. 8. Kushnick SD, Pelton-Henrion K, McCormick SA, Kimmelman CP. A scanning electron microscopic study of smoking and age-related changes in human nasal epithelium. Am J Rhinol 1992; 6:185–190. 9. Patterson CN. The aging nose: characteristics and correlation. Otolaryngol Clin N Am 1980; 13:275–288. 10. Hwang WS, Li B, Jin LH, Ngo K, Schachar NS, Hughes GN. Collagen fibril structure of normal aging and osteoarthritic cartilage. J Pathol 1992; 167:425–433. 11. Roughley PJ, Nguyen Q, Mort JS. Mechanisms of proteoglycan degradation in human articular cartilage. J Rheumatol 1991; 18(suppl 27):52–54. 12. Sakakura Y, Ukai K, Majima Y, Murai S, Harada T, Miyoshi Y. Nasal mucociliary clearance under various conditions. Acta Otolaryngol 1983; 96(1–2):167–173. 13. Himi T, Yoshioka I, Kataura A. Influence of age on the production of interleukin-8-like chemokine (GRO/CINC-1) in rat nasal mucosa. Eur Arch Otorhinolaryngol 1997; 254(2): 101–104. 14. Mabry RL. Nasal stuffiness due to systemic medications. Otolaryngol Head Neck Surg 1983; 91(1):93–94. 15. Sisson JH, Yonkers AJ, Waldman RH. Effects of guaifenesin on nasal mucociliary clearance and ciliary beat frequency in healthy volunteers. Chest 1995; 107(3):747–751. 16. Papsin B, McTavish A. Saline nasal irrigation: its role as an adjunct treatment. Can Fam Phys 2003; 49:168–173. 17. Nuutinen J, Holopainen E, Haahtela T, Ruoppi P, Silvasti M. Balanced physiological saline in the treatment of chronic rhinitis. Rhinology 1986; 24(4):265–269. 18. Bachmann G, Hommel G, Michel O. Effect of irrigation of the nose with isotonic salt solution on adult patients with chronic paranasal sinus disease. Eur Arch Otorhinolaryngol 2000; 257(10):537–541. 19. Rabago D, Zgierska A, Mundt M, Barrett B, Bobula J, Maberry R. Efficacy of daily hypertonic saline nasal irrigation among patients with sinusitis: a randomized controlled trial. J Fam Pract 2002; 51(12):1049–1055. 20. Tomooka LT, Murphy C, Davidson TM. Clinical study and literature review of nasal irrigation. Laryngoscope 2000; 110(7):1189–1193.

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21. Suman JD, Laube BL, Dalby R. Nasal nebulizers versus aqueous nasal spray pumps: a comparison of deposition patterns in human volunteers. Resp Drug Deliv 1998; 6:211–218. 22. Miller TR, Muntz HR, Gilbert ME, Orlandi RR. Comparison of topical medication delivery systems after sinus surgery. Annual meeting of the Western Section of the Triologic Society, Indian Wells, Ca., Jan 31–Feb 2, 2003. 23. Seppey M, Schweri T, Hausler R. Comparative randomized clinical study of tolerability and efficacy of Rhinomer Force 3 versus a reference product in postoperative care of the nasal fossae after endonasal surgery. ORL J Otorhinolaryngol Relat Spec 1996; 58(2): 87–92. 24. Heatley DG, McConnell KE, Kille TL, Leverson GE. Nasal irrigation for the alleviation of sinonasal symptoms. Otolaryngol Head Neck Surg 2001; 125(1):44–48. 25. Anglen JO, Apostoles S, Christensen G, Gainor B. The efficacy of various irrigation solutions in removing slime-producing Staphylococcus. J Orthop Trauma 1994; 8:390–396. 26. Olson DE, Rasgon BM, Hilsinger RL Jr. Radiographic comparison of three methods for nasal saline irrigation. Laryngoscope 2002; 112(8 Pt 1):1394–1398. 27. Grossan M. A device for nasal irrigation. Trans Am Acad Ophthalmol Otolaryngol 1974; 78(4):ORL279–280. 28. Mangete EDO, West D, Blankson CD. Hypertonic saline solution for wound dressing. Lancet 1992; 340:1351. 29. Talbot AR, Herr TM, Parsons DS. Mucociliary clearance and buffered hypertonic saline solution. Laryngoscope 1997; 107(4):500–503. 30. Shoseyov D, Bibi H, Shai P, Shoseyov N, Shazberg G, Hurvitz H. Treatment with hypertonic saline versus normal saline nasal wash of pediatric chronic sinusitis. J Allergy Clin Immunol 1998; 101(5):602–605. 31. Greiff L, Andersson M, Wollmer P, Persson CG. Hypertonic saline increases secretory and exudative responsiveness of human nasal airway in vivo. Eur Respir J 2003; 21(2): 308–312. 32. Adam P, Stiffman M, Blake RL Jr. A clinical trial of hypertonic saline nasal spray in subjects with the common cold or rhinosinusitis. Arch Fam Med 1998; 7(1):39–43. 33. Boek WM, Keles N, Graamans K, Huizing EH. Physiologic and hypertonic saline solutions impair ciliary activity in vitro. Laryngoscope 1999; 109(3):396–399. ¨ nal M, Gorur K, Ozcan C. Ringer-lactate solution versus isotonic saline solution on 34. U mucociliary function after nasal septal surgery. J Laryngol Otol 2001; 115(10):796–797. 35. Simmons JE, Richarson SD, Speth TF, et al. Development of a research strategy for integrated technology-based toxicological and chemical evaluation of complex mixtures of drinking water disinfection byproducts. Environ Health Perspect 2002; 110(suppl 6): 1013–1024. 36. Homer JJ, England RJ, Wilde AD, Harwood GRJ, Stafford ND. The effect of pH of douching solutions on mucociliary clearance. Clin Otolaryngol 1999; 24:312–315. 37. Davidson TM, Murphy C, Mitchell M, Smith C, Light M. Management of chronic sinusitis in cystic fibrosis. Laryngoscope 1995; 105(4 Pt 1):354–358. 38. Murphy C, Davidson TM, Jellison W, et al. Sinonasal disease and olfactory impairment in HIV disease: endoscopic sinus surgery and outcome measures. Laryngoscope 2000; 110(10 Pt 1):1707–1710. 39. Desrosiers MY, Salas-Prato M. Treatment of chronic rhinosinusitis refractory to other treatments with topical antibiotic therapy delivered by means of a large-particle nebulizer: results of a controlled trial. Otolaryngol Head Neck Surg 2001; 125(3):265–269. 40. Dudley JP. Atrophic rhinitis: antibiotic treatment. Am J Otolaryngol 1987; 8(6):387–390. 41. Ricchetti A, Landis BN, Maffioli A, Giger R, Zeng C, Lacroix J. Effect of anti-fungal nasal lavage with amphotericin B on nasal polyposis. J Laryngol Otol 2002; 116:251–263. 42. Rubenfeld GD, Clark BD, Candage R, Kass ES. A novel approach to invasive fungal sinusitis-use of adjuvant topical and nebulized amphotericin B. Annual meeting of the Triological Society, Naples, Fl., Jan 9–11, 2003.

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43. Sim MK. Treatment of disease without the use of drugs. VI. Treatment of rhinitis by a yogic process of cleaning and rubbing the nasal passage with a rubber catheter. Singapore Med J 1981; 22(3):121–123. 44. Johnsen J, Bratt B, Michel-Barron O, Glennow C, Petruson B. Pure sesame oil vs. isotonic sodium chloride solution as treatment for dry nasal mucosa. Arch Otolaryngol Head Neck Surg 2001; 127:1353–1356.

21 Epistaxis in the Geriatric Population Dana S. Smith and Mark K. Wax Department of Otolaryngology/Head and Neck Surgery, Oregon Health and Science University, Portland, Oregon, U.S.A.

INTRODUCTION Epistaxis is a problem that the otolaryngologist commonly encounters in the office. Although epistaxis occurs in all age groups, its incidence increases sharply in the elderly (1). The common usage of medications in the elderly, which impair coagulation, such as aspirin and warfarin, may contribute both to the high incidence and severity of the problem. Several local factors, such as dry nasal mucosa, stiff vessels from arteriosclerosis, and nasal trauma from frequent falls also contribute to the higher incidence in older individuals. While management in younger individuals is usually straightforward and in an office setting, the geriatric population typically presents to the emergency room and requires more complex treatment and/or procedures. This chapter will start with a description of the anatomy of the nasal cavity followed by a discussion of the otolaryngological management of epistaxis in the elderly.

ANATOMY The blood supply of the nasal cavity is derived from the internal and external carotid arteries. The internal carotid artery gives rise to the ophthalmic artery, which divides into a number of branches within the orbit, including the posterior and anterior ethmoid arteries. The posterior ethmoid artery exits the orbit through the posterior ethmoid foramen to cross medially along the roof of the ethmoid cavity to the anterior cranial fossa. It then passes through the cribriform plate and divides into septal and lateral nasal wall branches. These supply the posterior superior part of the septum in the back of the nose. The anterior ethmoid artery takes a similar route, traveling through the anterior ethmoid foramen, then crossing medially along the roof of the ethmoid cavity to the anterior cranial fossa. It passes through the cribriform plate before dividing into septal and lateral nasal wall branches. These branches supply the superior anterior part of the nose. This blood supply is important to understand, since the area of concern is usually high up in the nose and not accessible to conservative treatment. The intracranial course and relationship to the optic nerve 239

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are important in the surgical management. Management of bleeding from these sources is usually via an external approach. The external carotid artery gives rise to a number of branches including the facial artery and the internal maxillary artery. The facial artery branches include the superior labial artery, which in turn supplies the caudal septum via a septal branch. The internal maxillary artery divides into multiple terminal branches within the pterygopalatine fossa, including the sphenopalatine and descending palatine arteries. The sphenopalatine artery branches near the sphenopalatine foramen. One branch supplies the turbinates and lateral nasal wall, while the other crosses the face of the sphenoid sinus and supplies the nasal septum. The descending palatine artery travels through the greater palatine foramen, becoming the greater palatine artery. This continues anteriorly along the hard palate, then through the incisive foramen to supply the anterior septum. Kisselbach’s plexus (also known as Little’s area) is situated at the anterior nasal septum and consists of the anastamosis of the terminal branches from the several arteries supplying the nasal cavity. It includes the anterior and posterior ethmoids, the sphenopalatine, greater palatine, and superior labial septal branch. This area is especially significant since it is the most common site of epistaxis.

ETIOLOGY As in the overall population, epistaxis in older patients has multiple causes. These may be divided into local, medical, and pharmaceutical causes. It is important to note that multiple factors often contribute simultaneously to an episode of epistaxis, such that management is often more difficult in the elderly. For instance, epistaxis secondary to trauma in a younger patient may be easily managed, while the same problem in an elderly patient with hypertension and dry mucosa secondary to medication use is much more challenging.

Local Causes Mechanical causes are often responsible for mucosal injury leading to epistaxis. The elderly have a high rate of falls, many of which lead to nasal trauma. Even in the absence of a nasal fracture, mucosal lacerations may occur and result in profuse bleeding. In addition, many medical procedures such as the introduction of a nasogastric tube or feeding tube through the nasal cavity may disrupt the delicate mucosa of the septum or turbinates. Any surgical procedure involving the nose, such as functional endoscopic sinus surgery, may lead to epistaxis. The elderly often have dry mucous membranes, frequently as a result of generalized dehydration. Many medications may contribute to this. Dryness is also common in patients with general functional impairment such as residents of assisted living facilities. In addition, the common use of oxygen by nasal cannulas, often without the addition of humidification, may desiccate the nasal mucosa. Dry nasal mucosa can be fragile and prone to significant bleeding, even from seemingly minor mucosal trauma. Digital trauma (nose picking) is a frequently overlooked cause of epistaxis, which, by its repetitive nature, fuels the epistaxis/trauma cycle. Occasionally, despite a diligent search, epistaxis may start without any evident immediate cause.

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Medical Causes Many medical conditions, such as hypertension and various deficiencies in coagulation that include liver disease, are encountered in older patients and may contribute to epistaxis. Although hypertension has not been shown by itself to lead to a greater incidence of nosebleeds, it is far more prevalent in older patients, and may contribute to the severity and persistence of epistaxis; medical attention is required because the higher pressure bleeding vessels are less likely to spontaneously coagulate (2–4). Active epistaxis at presentation to the emergency department has been associated with hypertension (5). Exacerbating this is the effect of the ‘‘invasive treatment’’ on the blood pressure. Use of vasoconstrictors, tension, and stress all contribute to an increase in blood pressure and more bleeding. It is especially important to control blood pressure in order to control epistaxis in the acutely bleeding hypertensive patient. Coagulation deficiencies are common in the geriatric population as a result of the frequency of systemic diseases, which affect coagulation. Liver diseases such as hepatitis B, hepatitis C, and alcoholic cirrhosis may cause a decrease in production of coagulation factors. In the seriously ill patient, disseminated intravascular coagulation leads to a consumption of circulating coagulation factors, and may manifest as epistaxis. In addition, platelet disorders from systemic disease, such as thrombocytopenia from osteogenic malignancy or myelodysplasia, may cause epistaxis. Hereditary hemorrhagic telangectasia (also known as Osler–Weber–Rendu Syndrome) is another potential cause of epistaxis, which may be encountered in all age groups. Pharmaceutical causes of epistaxis are especially important to consider in the older population due to the multitude of medications taken by older patients that impair coagulation; these include aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), and warfarin, as well as platelet aggregation inhibitors. Increasingly, more patients are taking over-the-counter herbal medications, which may impair coagulation, such as gingko biloba. In the seriously ill hospitalized patient, heparin-induced thrombocytopenia, in which an autoimmune reaction consumes platelets, may cause epistaxis.

EVALUATION OF EPISTAXIS In order to effectively treat epistaxis, the location and the underlying etiology must be considered. A directed history should be taken. Although this does not need to be lengthy, especially if the patient requires rapid treatment for brisk bleeding, certain points should be elicited. The past medical history, including previous nosebleeds, bleeding disorders, liver disease, renal disease, and hypertension should be elucidated. The past surgical history, especially recent nasal operations such as endoscopic sinus surgery, should be obtained. The patient’s medications should be reviewed for the potential to impair coagulation. A family history of important disorders such as hemophilia and hereditary hemorrhagic telangectasia may be significant. Physical examination should include a directed head and neck examination for which the patient should be thoroughly prepared. A frank discussion and possible sedation is mandatory. All equipment needed for visualization and coagulation (cautery—either chemical or electrical) should be readily available. Cotton pledgets

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for nasal packing should be accessible and prepared in large numbers ahead of time. Anterior rhinoscopy should be performed with a nasal speculum, headlight, and Frazier tip suction. Often, this reveals the source of bleeding since most nosebleeds are located on the anterior septum. If the source of bleeding is still unclear, it is helpful to suction the nose and spray topical anesthetic (containing lidocaine and a vasoconstrictor) in order to perform rigid nasal endoscopy. The authors’ preference is a 1:1 mixture of 4% lidocaine and oxymetazoline. Endoscopy is then performed with a 2.7 mm 30 rigid nasal endoscope and Frazier tip suction. Transoral inspection of the posterior oropharynx is also performed to assess the extent of postnasal passage of blood from the nasal cavity. Laboratory examination includes a complete blood count, prothrombin time, and partial thromboplastin time in order to determine if excessive bleeding or coagulation deficiencies are present.

TREATMENT OF EPISTAXIS Conservative Measures Most episodes of epistaxis experienced by the elderly can be resolved with relatively simple measures. Often, a vasoconstrictor nasal spray such as oxymetazoline is sufficient to stop epistaxis (6). However, this should be used cautiously as many geriatric patients may become hypertensive, which may actually exacerbate bleeding. Silver nitrate cautery can also be used, and is most useful when the source of bleeding is on the anterior septum and the site is not briskly bleeding (6). Good preoperative vasoconstriction and anesthesia allow for placement of the silver nitrate stick. The bleeding site as well as the vessels leading to it should be cauterized. Silver nitrate should not be applied to the same site of both sides of the nasal septum due to the risk of septal perforation from devascularization of the cartilage and subsequent chondronecrosis. Absorbable cellulose sponges placed over the site may help with hemostasis and will protect the area while it heals from the cauterization. Unipolar electrocautery may also be used if the site is easily accessible via anterior rhinoscopy. Heat cautery may also be effective in this situation (7). This requires injection with topical anesthetic with epinephrine, which often slows the bleeding temporarily due to its vasoconstrictive effect. If the above measures fail, nasal packing is the next step (8,9). Packs provide hemostasis by their compressive effect. Packs come in a variety of materials, including preformed cellulose sponges, inflatable balloons, and strip gauze. Solid packs, such as balloon packs, will work best for anterior bleeding along the septum or medial aspect of the turbinates where the packs can directly compress the bleeding vessel. More posterior bleeding, often arising from the sphenopalatine arterial branches, may require anterior–posterior packing in order to provide adequate tamponade of the vessel (10). The posterior pack is positioned in the nasopharynx in order to give support to the anterior pack and to prevent it from falling into the nasopharynx. A gauze roll or Foley catheter may be used for the posterior pack. For the anterior pack, petroleum strip gauze layered from the nasal floor to the roof can be used. Often, bleeding in coagulopathic patients or hypertensive patients is difficult to control initially, so nasal packing may serve to slow or stop bleeding, while blood pressure is lowered and/or the coagulopathy corrected. Antibiotics with good Staphylococcus coverage are given to prevent bacterial overgrowth and possible toxic shock syndrome, and packs are generally removed after three days (11). Hypoxia

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and hypercarbia may also occur with packing in place, and monitoring with pulse oximetry may be warranted (12). In patients with several comorbidities, admission to the intensive care unit for observation may be necessary. Mucosal necrosis from pack pressure is another potential complication (13). Having anterior packs in place is relatively uncomfortable to the patient. If they are hypertensive, the placement of the packs and their continued presence will exacerbate the bleeding. Most patients will tolerate a unilateral pack once. If bilateral or repeated packing is required, then ancillary measures should be considered (14). Greater palatine artery injection, warm water irrigation of the nasal cavities, and fibrin glue application may also be effective in controlling epistaxis (9,15–17). Finally, it must be stressed that the hypertension and the high systolic pressure that is inevitably present must be controlled.

Considerations in Anticoagulated Geriatric Patients Deciding whether or not to correct the coagulopathy depends on the difficulty in controlling bleeding versus the risk of correcting the coagulopathy. For instance, a patient with atrial fibrillation and brisk bleeding may be corrected, as there is not a significantly elevated stroke risk for a short period of time; however, the risk of reversing coagulation in a patient with an artificial valve is significantly higher and may not be warranted unless the patient is having life-threatening epistaxis, which cannot be controlled by other measures. One small study demonstrated no advantage in reversing warfarin anticoagulation for control of epistaxis if the international normalized ratio (INR) was in therapeutic range (18). In these instances, use of an absorbable pack such as Gelfoam or Surgicel, which does not require removal, may be the best option.

Surgical Management of Recalcitrant Epistaxis The majority of epistaxis is controlled either by the patient in their home setting, in the physician’s office, or in the emergency room setting by local measures. Occasionally, patients will present with severe epistaxis that is not controllable with any of the simple measures mentioned above. It is difficult to get a grasp on the incidence of this intractable epistaxis, since the majority of centers that report on surgical or other invasive methodology for treatment of epistaxis are seeing a highly biased patient population. For instance, our institution has developed interest and expertise in angiographic embolization (19). All patients that present to our institution are so treated, yet we realize that this patient population is a small subset of the patients seen in the greater community. When medical management fails, patients are usually referred to the otolaryngologist for secondary procedures. Elderly patients who are hospitalized and fail more conservative management such as repeated nasal packing, blood transfusions, and control of comorbid medical conditions may require invasive procedures (10). The most common invasive procedures currently performed in the United States include internal maxillary artery ligation, angiographic embolization, anterior/ posterior sphenoid artery ligation, and external carotid artery ligation (19–22). Success rates for these options are similar, with the choice depending on the available expertise and/or experience in a particular institution.

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Angiographic Embolization The major cause of bleeding in the geriatric population is from branches of the internal maxillary artery. In the early 1970s, as angiographic techniques improved, various centers began to explore and utilize angiography in diagnosing, and ultimately treating epistaxis with embolization. This paradigm has continued with a number of centers obtaining expertise in the management of patients with this problem. A recent report from our institution describes 70 patients who were admitted with intractable epistaxis. All of the patients had been previously treated with nasal packing. A small minority underwent surgery or cauterization. A significant number (34%) had required blood transfusions prior to being admitted. Patients were admitted and brought to the angiography suite, where embolization of the internal maxillary arteries was performed. Epistaxis was controlled in >90% of patients, allowing for discharge within 24 hours. The embolization is carried out in the angiography suite under local anesthesia, and there is very little medical or local morbidity from the procedure itself (19). Complications, when they do occur, include pain, persistent bleeding, and neurologic events such as cerebrovascular accidents. In 10% of patients, epistaxis was not controlled with embolization, and operative intervention was required. Internal Maxillary Artery Ligation All that can be said for arteriographic embolization can be reiterated for surgical approaches (20). These require a general anesthetic for identification of branches of the internal maxillary artery in the pterygopalatine fossa, which are ligated with small hemoclips. While under anesthesia, an incision is made in the buccal mucosa overlying the maxillary sinus. The anterior and posterior walls of the maxillary sinus are removed. The pterygopalatine fossa is identified and the terminal branches of the internal maxillary arteries are identified and clipped. An endoscopic nasal approach may also be used to identify and clip branches of the internal maxillary artery. Reports of success, as well as morbidity, for this technique are identical to that for embolization with success rates between 85% and 95%. Patients are often discharged within 24 hours. In skilled surgical hands, this technique carries little local morbidity and has excellent short- and long-term control rates. Patients who fail this procedure do so because the bleeding is from the anterior or posterior ethmoid arteries or small unidentified branches. Continued bleeding from these small branches can be handled with angiographic embolization. The incidence of local complications due to maxillary sinus violation, as well as numbness secondary to the infraorbital nerve traction, runs upward to 25%. Anterior/Posterior Ethmoid Artery Ligation Occasionally, one encounters a patient whose epistaxis originates high in the nasal vault. These areas are supplied by the anterior or posterior ethmoid arteries. Bleeding in this area is difficult to visualize endoscopically. Obtaining sufficient pressure with a nasal pack is problematic in an awake patient. Since the origin of these vessels is from the internal carotid, embolization of the internal maxillary artery will not control the bleeding. Embolization through the internal carotid system is not undertaken due to the risk of intracranial complications and blindness. Unfortunately, the diagnosis of bleeding from these vessels is often that of exclusion. Most patients who

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require intervention due to bleeding from these arteries have either failed angiographic embolization or some other surgical approach. It is difficult to anatomically pinpoint these vessels during a physical or endoscopic examination. The technical approach is through a small incision in the medial canthus of the eye, along the lamina papyracea. The anterior and posterior ethmoid arteries are anatomically in a very predictable location, and technically the procedure is easy to perform. Particular care must be exercised in approaching the posterior ethmoid artery because of its proximity to the orbital nerve. The morbidity of the surgical procedure, other than the general anesthetic, is low, and control rates for epistaxis have been reported in >90% of cases (21). External Carotid Artery Ligation The external carotid artery is the feeding vessel for the internal maxillary artery, as well as the facial artery. Thus, ligation of the external carotid artery should devascularize the ipsilateral nasal cavity. Flow from collaterals, as well as connections from the contralateral side, will prevent tissue necrosis. Success rates with external carotid artery ligation are reported in up to 50% of patients (22). The same factors that prevent soft tissue necrosis on the ipsilateral side also contribute to failure of this procedure. Collateral flow from the contralateral side, as well as from intracranially, will lead to failure in controlling the epistaxis. This method is rarely undertaken today because of low success rates and the availability of superior surgical, angiographic, and endoscopic techniques. Endoscopic Sphenopalatine Artery Ligation Recently, a small number of centers have begun to explore the use of endoscopic sinus surgical techniques for control of epistaxis (23). The majority of posterior epistaxis is felt to be due from branches of the sphenopalatine artery that are branches of the internal maxillary artery. Endoscopic sinus surgery, which is a technical approach to sinus diseases, has seen an explosion in popularity due to improvements in both technique and instrumentation. With this has come an ability to approach the foramen of the sphenopalatine artery in the posterior aspect of the nose. The nose is approached as for an endoscopic nasal procedure. Landmarks consist of the middle turbinate and basal lamella. In this area, a small mucosal flap is elevated and posterior dissection is undertaken to identify the foramen and the sphenopalatine artery. Once it has been visualized it is clipped. Rarely is it necessary to pack the nose. Postoperative care is as for any patient who has undergone endoscopic sinus surgery. A small number of centers have reported expertise with patients who have had recalcitrant epistaxis and had the sphenopalatine artery ligated through a transnasal endoscopic approach. As with most other surgical techniques, success rates in the authors’ hands have been in the 90% range. As more and more centers, as well as otolaryngologists, are trained in this method, the low morbidity and ease of the procedure may see the emergence of this new technique as the preferred treatment for recalcitrant epistaxis. SUMMARY While medical management of epistaxis is satisfactory in the majority of cases, in patients where simple medical means do not control the bleeding, surgical intervention

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should be considered. Several surgical approaches are available for the treatment of recalcitrant epistaxis, and the choice depends on the experience and preference of the attending surgeon. Success rates are high for all methods in well-trained hands.

REFERENCES 1. Tomkinson A, Roblin DG, Flanagan P, Wunie SM, Backhouse S. Patterns of hospital attendance with epistaxis. Rhinology 1997; 35(3):129–131. 2. Fasce E, Flores M, Fasce F. Prevalence of symptoms associated with blood pressure in normal and hypertensive population. Revista Medica de Chile 2002; 130(2):160–166. 3. Lubianca-Neto JF, Bredemeier M, Carvalhal EF, et al. A study of the association between epistaxis and the severity of hypertension. Am J Rhinol 1998; 12(4):269–272. 4. Mendelson G, Ness J, Aronow WS. Drug treatment of hypertension in older persons in an academic hospital-based geriatrics practice. J Am Geriatr Soc 1999; 47(5):597–599. 5. Herkner H, Havel C, Mullner M, et al. Active epistaxis at ED presentation is associated with arterial hypertension. Am J Emergency Med 2002; 20(2):92–95. 6. Krempl GA, Noorily AD. Use of oxymetazoline in the management of epistaxis. Ann Otol Rhinol Laryngol 1995; 104(9 Pt 1):704–706. 7. Quine SM, Gray RF, Rudd M, von Blumenthal H. Microscope and hot wire cautery management of 100 consecutive patients with acute epistaxis—a superior method to traditional packing. J Laryngol Otol 1994; 108(10):845–848. 8. Pringle MB, Beasley P, Brightwell AP. The use of Merocel nasal packs in the treatment of epistaxis. J Laryngol Otol 1996; 110(6):543–546. 9. Stangerup SE, Dommerby H, Lau T. Hot-water irrigation as a treatment of posterior epistaxis. Rhinology 1996; 34(1):18–20. 10. Shaw CB, Wax MK, Wetmore SJ. Epistaxis: a comparison of treatment. Otolaryngol Head Neck Surg 1993; 109:60–65. 11. Aeumjaturapat S, Supanakorn S, Cutchavaree A. Toxic shock syndrome after anteriorposterior nasal packing. J Med Assoc Thailand 2001; 84(3):453–458. 12. Hady MR, Kodeira KZ, Nasef AH. The effect of nasal packing on arterial blood gases and acid–base balance and its clinical importance. J Laryngol Otol 1983; 97(7):599–604. 13. Uslu SS, Ileri F, Koybasioglu A, Celik H, Sargon M, Ozbilen S. Scanning electron microscopy of hydroxylated polyvinyl acetal and conventional gauze strip nasal packing materials. Am J Rhinol 2001; 15(2):91–94. 14. Monte ED, Belmont MJ, Wax MK. Management paradigms for posterior epistaxis: a comparison of costs and complications. Otolaryngol Head Neck Surg 1999; 121:103–106. 15. Bharadwaj VK, Novotny GM. Greater palatine canal injection: an alternative to the posterior nasal packing and arterial ligation in epistaxis. J Otolaryngol 1986; 15(2):94–100. 16. Vaiman M, Segal S, Eviatar E. Fibrin glue treatment for epistaxis. Rhinology 2002; 40(2):88–91. 17. Walshe P, Harkin C, Murphy S, Shah C, Curran A, McShane D. The use of fibrin glue in refractory coagulopathic epistaxis. Clin Otolaryngol Allied Sci 2001; 26(4):284–285. 18. Srinivasan V, Patel H, John DG, Worsley A. Warfarin and epistaxis: should warfarin always be discontinued? Clin Otolaryngol Allied Sci 1997; 22(6):542–544. 19. Christensen NP, Smith DS, Barnwell SL, Wax MK. Arterial embolization in the management of posterior epistaxis. Presented at the Western Section of Triologic Society, January 31, 2004, Otolaryngol Head Neck Surg. Submitted March 2004. 20. Strong EB, Bell DA, Johnson LP, Jacobs JM. Intractable epistaxis: transantral ligation vs. embolization: efficacy review and cost analysis. Otolaryngol Head Neck Surg 1995; 113(6):674–678. 21. Douglas SA, Gupta D. Endoscopic assisted external approach anterior ethmoidal artery ligation for the management of epistaxis. J Laryngol Otol 2003; 117(2):132–133.

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22. Spafford P, Durham JS. Epistaxis: efficacy of arterial ligation and long-term outcome. J Otolaryngol 1992; 21(4):252–256. 23. Kumar S, Shetty A, Rockey J, Nilssen E. Contemporary surgical treatment of epistaxis. What is the evidence for sphenopalatine artery ligation? Clin Otolaryngol 2003; 28(4):360–363.

PART III: ORAL DISORDERS

22 Oral Mucosal Pathology in the Geriatric Population Bobby M. Collins Oral Medicine and Pathology, University of Pittsburgh School of Dental Medicine, Pittsburgh, Pennsylvania, U.S.A.

Mucosal diseases are relatively common in the geriatric population. Systemic diseases that compromise liver or renal function can contribute to mucositis, and any number of medications taken for chronic disease can affect the integrity of the oral mucosa. Xerostomia, epithelial atrophy, ulceration, and vesiculation are some of the more common oral findings associated with medication usage. Radiation therapy, chemotherapy, drug ‘‘allergy,’’ and paraneoplastic syndromes are reported to cause clinical mimics (such as desquamation) to a number of mucocutaneous diseases discussed in this chapter. Vesiculobullous diseases, though rare, are problematic for both patient and practitioner. Patients suffer through bouts of oral pain that worsen with the consumption of hot, spicy foods and acidic beverages. Speech and oral hygiene are compromised when desquamation or ulceration occur. Effective treatment relies upon an accurate diagnosis. Some of the more common oral diseases associated with ulceration are: herpetic stomatitis, erythema multiforme, lichen planus, pemphigoid, pemphigus, and aphthous ulcers. Aphthous ulcers do not pass through a vesicular stage. They simply arise as ulcers and do not represent vesiculobullous disease. Their discussion here is due to common occurrence and frequent confusion with vesiculobullous disease. While the clinical presentation will be similar in a number of the vesiculobullous diseases, duration and distribution often differ, as well as the presence of associated mucosal and cutaneous changes in other anatomic areas. Desquamative gingivitis (Fig. 1) is a clinical description of the gingival sloughing seen in erosive lichen planus, pemphigoid, pemphigus, and as a reaction to certain ingredients in toothpastes (pyrophosphates, peroxides, surfactants, and preservatives). The virally induced vesiculobullous lesions are often so characteristic as to make biopsy unnecessary. A clinical diagnosis is made. However, the majority of the vesicular lesions require a representative biopsy. The excised tissue is divided and submitted in 10% neutral buffered formalin for hematoxylin and eosin staining (H and E), and the remaining tissue is placed in phosphate-buffered saline (Michel’s solution) or Zeus1 tissue fixative for immunofluorescence studies. In the submitted 249

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Figure 1 Clinical photo of maxillary and mandibular gingival erosions or ‘‘desquamative gingivitis’’ in a patient with mucous membrane pemphigoid.

tissues, the epithelium must be attached to the underlying connective tissue. As the tissue is fragile, this requires careful tissue manipulation on the part of the surgeon performing the biopsy. HERPES SIMPLEX The most common of the vesiculobullous diseases are virally related. Herpes simplex virus (HSV) antibodies are estimated to be as high as 80–85% in the general population. While the initial infection is largely asymptomatic, about 30% of those infected may present with primary herpetic gingivostomatitis. The clinical features of malaise, cervical lymphadenopathy, fever, and oral pain are coincident with clustered vesicular eruptions perinasally, periorally, and along the lips and mucosa. Fiery red gingiva are seen on intraoral examination. Oral discomfort will limit eating, speaking, and adequate oral hygiene. Resolution occurs in 7 to 14 days, with the virus traveling in a retrograde fashion to the sensory ganglion, where it lies dormant until reactivation (viral latency). Each vesicle has about a three-day life cycle, with new vesicle eruptions occurring synchronously, until immunosurveillance eventually controls the infection in about two weeks. Two viral forms exist, with HSV I affecting the oral region and HSV II affecting the genital region. However, the forms may exist at either site. Both forms may coexist, as HSV I provides minimal immunologic protection for infection with HSV II. HSV II infection outbreaks tend to persist for longer than 14 days. The age of presentation varies from 5 to 10 years, but any previously unexposed person can present with the primary infection. Recurrences commonly are noted periorally and are heralded by a tingling, itching, or burning sensation as the virus travels from the sensory ganglion down the sensory nerve. The resulting viremia causes infection or reinfection of keratinized mucosal epithelium. Intraorally, the herpetic lesions are seen as clustered, punctate shallow ulcerations on the attached

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gingiva and palate. Fragile vesicles coalesce, burst, and ulcerate in an area coinciding with the initial infection. The vesicle fluid is highly contagious, and scratching may subsequently autoinnoculate other anatomic sites such as the eyes or genitalia. The ulcers are shallow with a punctate round appearance commonly, but trauma and coalescence can produce large ulcers with ragged, irregular borders. Vesicles that burst are responsible for the amber brown crusts that are a common sight in recurrent herpes labialis. Histologically, ballooning degeneration and keratinolysis are observed. The lytic cells merge and fuse to produce giant cells. The nuclei of adjacent cells merge and show nuclear molding. These nuclei are large and have a ‘‘smudged’’ or ‘‘ground glass’’ appearance. Viral inclusions are seen within the nuclei and are called Lipshu¨tz bodies or Cowdry type A inclusions. L-lysine consumption, topical vitamin A application, and ice serve as anecdotal preventives and possibly palliatives, but antiviral medications are more effective in treatment of the disease. Abreva1 (doconasol cream 10%) is available over the counter and is reported to accelerate healing time. Denavir1 (pencyclovir), available by prescription, is slightly more effective than Zovirax1 (acyclovir). Valtrex1 (valacyclovir), which is prescribed for genital herpes, has also been used for oral herpes simplex and herpes zoster infections. Other viral infections are more rare than herpes simplex. Coxsackie viral outbreaks are seasonal and commonly occur in clusters of school-aged children. In herpangina (caused by Coxsackie group A), vesicles and shallow ulcers are noted on the soft palate and tonsillar complex. The soft palate, tonsillar area, buccal mucosa, and palmar and plantar skin are affected in hand, foot, and mouth disease (Coxsackie A 12). Varicella zoster virus gives rise to ‘‘chicken pox’’ in children as the initial or primary infection, and to herpes zoster or ‘‘shingles’’ in older adults as the latent disease.

ERYTHEMA MULTIFORME Erythema mutiforme (EM) is an acute vesiculoulcerative disease characterized by bloody crusting of mucosal surfaces. The disease is most common in young adults, and males are affected more often than females. Erythema multiforme is self-limiting and is thought to be a type of hypersensitivity reaction to certain drugs and infections. Drugs like sulfonamides and barbiturates, as well as preceding infections by herpes simplex virus and Mycoplasma pneumonia, are considered precipitating factors. There is a spectrum of disease that may be as mild as shallow oral ulceration and bloody crusting of the lips, or as severe as the bloody crusting of the oral mucosa, lips, eyes, and genitalia of Stevens–Johnson syndrome. The most severe form is life threatening. It shows sloughing of large segments of skin and mucosal surfaces, and is called toxic epidermal necrolysis or Lyell’s disease. This form is commonly associated with medication ingestion (antibiotics, analgesics, antihypertensives, and antianginals). The oral ulcerations of erythema multiforme are sudden in onset and sufficiently painful to limit eating and drinking. Topical anesthetics and supportive care are necessary to ensure adequate hydration and nutrition. When EM follows recurrent herpetic eruptions, consideration must be given to treating the preceding herpes infection to prevent the EM outbreak. The skin lesions of EM show central erythema and a pale ring at the periphery resembling a ‘‘bullseye’’ or ‘‘iris.’’ Histologically, necrotic keratinocytes and epithelial edema are noted. Edematous separation may be intraepithelial or subepithelial, and a nonspecific inflammatory

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infiltrate is present. The clinical presentation is distinctive enough to render a diagnosis. Corticosteroids are used in the management of EM, but care must be taken if there was a preceding herpetic infection. The use of corticosteroids for the treatment of Stevens–Johnson syndrome or toxic epidermal necrolysis is controversial, as some cases are linked to a previous or coincident infection. The possibility of secondary or superinfection is considered a great risk. However, treatment success with controlled trials of corticosteroid therapy has been reported.

PEMPHIGOID Pemphigoid is an autoimmune disease that occurs in older adults, with female occurrence about 2:1 over males. The mean age of presentation is 60 years. There are two forms of the disease: (i) bullous pemphigoid, which is largely a cutaneous disease causing tense bullae that rarely affects the oral mucosa, and (ii) mucous membrane or cicatricial pemphigoid that commonly has oral lesions as the initial presentation. In mucous membrane pemphigoid, any mucosal surface can be affected, including soft palate, buccal mucosa, and gingiva. The bullae may present with straw-colored fluid, or they may be hemorrhagic. The bullae are fragile and rupture easily, giving rise to irregular erosions with a gray-white coating in areas representing sloughed, necrotic surface epithelium. These erosions may be chronic and are accompanied by slow healing and often scarring. The areas of desquamation are indistinguishable from those of pemphigus. Eye lesions in mucous membrane pemphigoid often scar down, leaving a fibrous attachment between the globe and eyelid. This is called symblepheron (or ankyloblepheron, if complete) and is the reason patients diagnosed with mucous membrane pemphigoid should see an ophthalmologist. Immunologic attack occurs on antigenic proteins in the basement membrane zone leading to a subepithelial separation. Histologically, the epithelium separates cleanly from the lamina propria and maintains its rete peg architecture (Fig. 2A and B). Immunofluorescent stains [immunoglobulin G (IgG), C3, and to a lesser extent, IgA] decorate the attachment interface between the epithelium and the lamina propria/connective tissue, thus highlighting the area of immunologic attack. Pemphigoid is treated with steroids and steroid-sparing agents (dapsone, cyclophosphamide), tetracycline, and niacinamide, and more recently tacrolimus and intravenous immunoglobulin. Pemphigoid has a good prognosis with occasional long periods of remission. Dapsone usage requires frequent assessment of liver function and hematologic reactions to evaluate hemolysis and methemoglobulinemia.

PEMPHIGUS Pemphigus is an uncommon autoimmune acantholytic disease in which oral lesions are often the presenting feature. The average age of onset is 49 years. Many clinical forms exist, but pemphigus vulgaris is the most severe form and will be discussed here. The disease occurs most often in middle-aged women and those of Eastern European Jewish ancestry. Extensive bullous eruptions involve the skin and mucosal surfaces and rupture to yield ragged ulcers. The remnants of surface epithelium are graywhite and necrotic and, unlike pemphigoid, do not tend to heal without treatment. Pemphigus is a serious disease, and prior to the advent of steroid therapy, survival averaged about two years.

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Figure 2 (A) Mucous membrane pemphigoid: H and E stained slide demonstrates sub-basilar separation of the epithelium in pemphigoid. Direct immunofluorescence will demonstrate autoimmune attack along the basement membrane zone. (B) Diagram of sub-basilar discohesion resulting from immunologic attack on the hemidesmosomes in the basement membrane zone.

Histologically, pemphigus characteristically shows acantholysis or epithelial separation within the spinous layer (Fig. 3A and B). The immunologic attack occurs on desmogleins that serve as intercellular attachment proteins. Acantholytic cells float free of contact inhibition and spread out to resemble ‘‘fried eggs,’’ and are called Tzanck cells. The basal cell layer remains adherent to the connective tissue and resembles ‘‘tombstones.’’ Tzanck cells are noted between the adherent basal cells and the superficial epithelium. Immunofluorescence decorates antigenic desmosomal proteins between the epithelial cells. The basal cells remain adherent, because they lack the particular desmoglein that serves as the autoantigen.

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Figure 3 (A) Pemphigus vulgaris: An H and E stained slide demonstrates suprabasilar acantholysis due to dissolution of intercellular bridges in pemphigus. Direct immunofluorescence will highlight the intraepithelial immunologic attack. (B) Diagram of epithelial discohesion or suprabasilar acantholysis resulting from immunologic attack on desmosomal desmogleins.

The treatment for pemphigus is long-term steroid therapy, or therapy with steroidsparing agents (azathioprine). This has vastly improved the formerly dismal prognosis. Promising new drugs for the treatment of pemphigus include intravenous immunoglobulin and mycophenolate. LICHEN PLANUS Lichen planus (LP) is a common mucocutaneous disease. Although the cause is unknown, its chronic nature and prevalence in females is suggestive of an autoimmune

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process. Adults over the age of 35 are most often affected, and the disease is largely asymptomatic. Those affected are commonly unaware of the condition until it is brought to their attention by a dentist, hygienist, or other health care provider during an oral exam. Symptoms exist in the erosive form of disease or when there is a superimposed fungal infection. Lichen planus and lichenoid reactions are exacerbated by contact with amalgam restorations and are noticed frequently in the buccal mucosa that is in contact with the restoration. The classic appearance of oral lichen planus is radiating white lines that intersect in a ‘‘lacy’’ pattern most noticeable in the buccal mucosa (Fig. 4). These Wickham’s striae are also seen on the ventral tongue. The dorsal tongue shows white plaque-like lesions when affected by lichen planus. The gingiva show desquamation and shallow ulceration, commonly along the tips of the papillae. Lichen planus is the most common cause of desquamative gingivitis because LP is more prevalent than pemphigoid. Bullous lesions and ulceration are known to occur with LP. These clinical features may represent the erosive form of the disease. Although it is controversial, erosive lichen planus is felt to have the greatest potential for malignant transformation at 1.2% to 1.4%. Histologically, hyperkeratosis is responsible for the appearance of Wickham’s striae. Vacuolar or hydropic degeneration of the epithelial basal cell layer is noted over a band-like, T-lymphocytic infiltrate and angular ‘‘sawtoothing’’ of the rete pegs can be seen. The sawtooth rete peg appearance is more common in cutaneous biopsies. The degeneration of the basal cell layer results in spinous cells approximating the basement membrane zone. This is referred to as squamatization. The T-cell lymphocytic infiltrate ‘‘hugs’’ the basement membrane zone. This is the characteristic histologic picture of interface mucositis. Gingival biopsies will contain abundant plasma cells.

Figure 4 Clinical photo: Buccal mucosal ulcer and radiating, white linear ‘‘Wickham’s striae’’ in a patient with lichen planus with ulceration.

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The treatment for symptomatic lichen planus includes topical ‘‘swish and spit’’ steroids, steroid gels, and systemic steroids depending on the severity. Topical tacrolimus and tetracycline with niacinamide also provide relief in erosive lichen planus. When lichen planus is symptomatic due to candidiasis, fluconazole is an effective therapy.

LICHENOID DRUG REACTION The clinical and histologic appearance of lichenoid drug reactions are identical to lichen planus. This striking similarity has caused some authorities to propose that lichen planus is a patterned response to various antigens rather than a specific disease. Numerous medications have been identified as causing the lichenoid eruption, and include nonsteroidal anti-inflammatory drugs, antimalarials, antibiotics, and antiarthritics. Allopurinol can precipitate erosive or ulcerative lichenoid lesions. These lesions can be extensive, and discontinuing the allopurinol provides relief in about two weeks. Amalgam restorations also exacerbate lichenoid lesions in mucosa in contact with the material. Removal of the dental amalgam can cause the lesions to resolve, but replacement dental materials may also precipitate the lichenoid reaction. The association of lichen planus and hepatitis C is more likely a lichenoid reaction to the interferon or ribavirin therapy for the hepatitis. A perusal of the patient’s medical and medication history can be quite beneficial in diagnosing lichenoid drug eruptions. Consultation with the patient’s primary care physician and a search of a drug information reference is both necessary and informative. Treatment of lichenoid drug reactions is simple when the suspected medication can be changed or suspended. However, in some cases, this is not possible without adverse systemic health effects. In this instance, topical steroid therapy is attempted. As in lichen planus, if erosions are not present, remember that a candidal infection can cause symptoms.

APHTHOUS ULCERS Aphthous ulcers are a common cause of oral discomfort found in 10–20% of the general population, but with a reported incidence as high as 40% in some populations (college students at exam time). The etiology is unknown, but is likely an immunologic irregularity. Aphthae exist in three forms: minor, major, and herpetiform or clusterform. Minor ulcers are the most common and account for 80% of the lesions. They have a regular round or oval border, surrounded by a fiery red halo, and are surfaced by a tan-gray pseudomembrane (fibrin clot). The ulcers are 4–6 mm in diameter, occur on loose, nonkeratinized mucosa, and are exquisitely painful for their size. One to 10 minor ulcers may be present, and there is a tendency for recurrence, therefore the clinical diagnosis of recurrent aphthous stomatitis. Each ulcer lasts from 7 to 10 days. The major ulcers are larger than 1 cm, typically single, and can extend onto keratinized tissue. These persist for four to six weeks and heal with scarring. Clusterform aphthae resemble the punctate ulcers of recurrent herpetic stomatitis. They are 2–3 mm in diameter and number from 10 to 100. They extend onto keratinized mucosa and into the oropharnyx and esophagus. AIDS and other immunocompromised patients are frequently affected with clusterform lesions. Each ulcer lasts 7 to 10 days, but the multiple ulcers account for disease persistence for a month or more.

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Histologically, aphthae are nonspecific ulcers with a surface fibrin clot. The clot overlies granulation tissue containing a mixed inflammatory infiltrate that is rich with lymphocytes and histiocytes. The peripheral epithelium is edematous. Aphthous ulcers or aphthous-like ulcers are also seen in systemic diseases, such as Crohn’s disease (a chronic granulomatous disease with ‘‘skip’’ lesions from mouth to anus) and Behc¸et’s disease (oral and genital ulcers and occular inflammation). Aphthous ulcers are treated with palliation or topical corticosteroids. Laser coagulation is said to immediately halt the discomfort with aphthae, but may cause scarring. Chemical cautery may cause scarring, and often the materials are toxic.

BIOPSY A representative biopsy can be a difficult task in vesiculobullous disease. First, a nonulcerated site must be located, and care must be taken on biopsy to ensure that the epithelium remains attached to the connective tissue. The clinician should obtain a positive Nikolsky sign, then biopsy the area adjacent to it. Using either the bow or straight surface of a dental explorer, rub lightly for about a minute. A positive Nikolsky sign is obtained when the epithelium slides over the underlying tissue. This separation and slide is due to the loss of epithelial attachment and edema created by immunologic attack on the cellular ‘‘cementing’’ proteins within the desmosomes in pemphigus and hemidesmosomes in pemphigoid. Look at the tissue color and surface texture to be sure that epithelial integrity exists (pink and stippled, as opposed to red, shiny, and easily bleeding). Anesthetize the area with block anesthesia, if possible, to avoid the edematous tissue distension that is obtained with infiltration. Place a black silk traction suture centrally within the tissue to be excised to assist in tissue adherence and ease of manipulation. Tissue forceps tend to crush and separate the tissues. With sharp scalpel dissection, remove an ellipse that is at least 5–6 mm in length, 4–5 mm in width, and 3 mm in depth. Scalpel biopsy is the least traumatic for this fragile tissue. Laser and electrosurgery generate heat and edema that hasten epithelial separation and mask or destroy significant microscopic architecture. The traction suture facilitates the excision laterally and deep. When the tissue is removed it is carefully divided to provide tissue for hematoxylin and eosin staining (H and E) and direct immunofluorescence staining. The tissue for H and E submission is placed in 10% neutral buffered formalin. The formalin fixation destroys tissue antigenicity, but prevents cellular degradation, preserving the cellular and tissue detail that existed at excision. The tissue for immunoflurescence is placed in a special solution that preserves tissue antigenicity, either Michel’s solution (a phosphate-buffered saline) or Zeus1 tissue fixative (N-ethylmaleimide ammonium sulfate and citrate buffer). These solutions are shelf-life sensitive and should be requested from your lab shortly before the surgical biopsy. If a white precipitate is present at the specimen jar lid, then the solution is not optimal for tissue transport. Direct immunofluorescence provides a method of determining the site of immunologic attack and assists in the diagnosis of pemphigus, pemphigoid, and erosive lichen planus, as well as many other immune-mediated vesiculobullous processes. The tissues are processed, sectioned, and reacted with a panel of antibodies to human immunoglobulins, fibrinogen, and C3. These antibodies are labeled or ‘‘tagged’’ with a dye (fluorescein). This dye will fluoresce under ultraviolet (UV) light reflected through the tissue on the microscopic slide, highlighting the site of attack. The common panel of antibodies is IgG, IgA, IgM, fibrinogen, and C3

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(a component of complement). When the tissues are examined with a special microscope equipped with a UV light source, the location and pattern of fluorescence determine the diagnosis. Pemphigus vulgaris shows deposition between the epithelial cells, as the attack is on the epithelial desmosomal cementing substances (desmogleins). IgG, C3, and IgA highlight or decorate more intensely between epithelial cells or interepithelially. In pemphigus, the basal cells adhere to the basement membrane and resemble ‘‘tombstones.’’ The interepithelial separation gives rise to acantholytic Tzanck cells that float in the edema fluid above the firmly attached basal cell layer. The split or separation is up and can be remembered by the presence of the letter ‘‘u’’ in pemphigus. Pemphigoid shows a linear pattern of fluorescence at the basement membrane zone. IgG and C3 stain more intensely in the area of epithelial attachment to the basement membrane zone. Epithelial rete pegs with an intact basal layer are preserved, and the lamina propria extensions are cleanly separated from the overlying epithelium. The split or separation is down and is remembered by the presence of the letter ‘‘d’’ in pemphigoid. Lichen planus shows a sub-basilar deposition of fibrinogen. The other antibodies are unreactive. Lichen planus is characterized by vacuolar degeneration of the basal cell layer, ‘‘sawtoothing’’ of the rete pegs, and a band-like T-lymphocytic infiltrate in the superficial lamina propria. SELECTED SUGGESTED MEDICATIONSa 1. 1-2-3 Mouthwash [30 mL of 2% viscous lidocaine, 60 mL of benadryl elixir (12.5 mg benadryl per 5 mL) and 90 mL Maalox]. Mix thoroughly. Dispense 180 cm3. Rinse with one teaspoonful for 30 seconds as needed for pain. Comment: Sterile water can be substituted for lidocaine if the patient does not desire profound numbness. To better remember the formulation, all ingredients can be mixed in equal amounts. This mouthrinse works well for traumatic ulcers, aphthous ulcers, and occasionally for sore and burning mouth as palliation until a definitive diagnosis is rendered. Aphthous Ulcers, Lichen Planus, Pemphigoid 1. Decadron elixir (dexamethasone elixir) 0.5 mg/5 mL. Dispense one pint bottle. Rinse with one teaspoonful for full two minutes, then expectorate; four times daily. Do not swallow. Do not eat or drink for 20 minutes after usage. 2. Prelone syrup (prednisolone) 15 mg/5 mL. Dispense one pint bottle. Rinse with one-third teaspoonful for full two minutes, then expectorate; four times daily. Comment: Works well for generalized ulcerations like aphthae. Can be used for symptomatic lichen planus. Use of a steroid preparation will make your patient prone to develop a fungal infection. Follow your patients regularly

a

Changes in disease treatment and drug therapy require vigilant monitoring of product information. One should always consult a drug reference for accuracy in dosing, administration, interactions, and contraindications.

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when they are using a steroid. A history of diabetes, peptic ulcer disease, osteoporosis, or tuberculosis will obviate systemic steroid use, but topical ‘‘swish and spit’’ preparations are tolerated. 3. Lidex gel (fluocinonide) 0.05%. Dispense one 15 g tube. Apply thin coat to oral ulcers up to QID. Comment: Works well for one or few ulcers. Warn patients about the potential bitter taste. Do not use on facial skin. Steroids will predispose to fungal infections.

Recurrent Herpetic Lesions 1. Denavir cream 1%. Dispense one 2 g tube. Apply thin coat to herpetic lesions every two hours for four days. Comment: Used to shorten the course of recurrent herpes simplex virus lesions of lips, commissure. Works best when application begins at first sign of tingling prodrome. 2. Valtrex (valacyclovir) 500 mg. Dispense eight tablets. Take four tablets when ‘‘tingling or itching’’ occurs, then four tablets 12 hours later. Do not take this medication for herpes labialis/oralis any longer than one day. Oral Candidiasis 1. Diflucan 100 mg tablets. Dispense 11 tablets. Take two tablets to start, then one tablet each morning until gone. Comment: Use of thiazide diuretics potentiates the effect of fluconazole. Severe oropharyngeal and esophageal candidiasis will require a two-week course. If there are frequent, recurrent candidal infections, immunosuppression or systemic disease should be suspected.

BIBLIOGRAPHY Anhalt G. Pemphigoid: bullous and cicatricial. Dermatol Clin 1990; 8:701. Aslanzadeh J, Heim K, Espy M, et al. Detection of HSV-specific DNA in biopsy tissue of patients with erythema multiforme by polymerase chain reaction. Br J Dermatol 1992; 126:19. Farthing PM, Maragou P, Coates M, et al. Characteristics of the oral lesions in patients with cutaneous recurrent erythema multiforme. J Oral Pathol Med 1995; 24:9. MacPhail L, Greenspan D. Herpetic gingivostomatitis in a 70-year old man. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79:50. Mydlarski PR, Mittman N, Shear NH. Intravenous immunoglobulin: use in dermatology. SkinTher Lett 2004; 9:1. Robinson JC, Lozada-Nur F, Frieden I. Oral pemphigus vulgaris: a review of the literature and a report on the management of 12 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997; 84:349.

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Scully C. Orofacial herpes simplex virus infections: current concepts in the epidemiology, pathogenesis and treatment, and disorders in which the virus may be implicated. Oral Surg Oral Med Oral Pathol 1989; 68:701. Vincent SD, Lilly GE, Baker KA. Clinical, historic, and therapeutic features of cicatricial pemphigoid: a literature review and open therapeutic trial with corticosteroids. Oral Surg Oral Med Oral Pathol 1993; 76:453.

23 Halitosis in the Elderly David E. Eibling Department of Otolaryngology—Head and Neck Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION One of the more embarrassing problems faced by any individual is an unacceptable odor that accompanies their person. Halitosis is the most ubiquitous of these odors, is common in individuals of all ages, and usually originates from within the oral cavity. Essentially every individual has some degree of ‘‘morning breath’’ upon arising prior to oral cleansing. Ingesting food and drink and/or formal oral care rapidly reduce the odor in most individuals. Oral odor may persist throughout the day and may be noted by the patient themselves or their families. Occasionally, detection of halitosis will prompt an evaluation by an otolaryngologist in a search for the cause as well as recommendations for therapy. Problems with objectionable odor are more often encountered in the elderly since many suffer from varying degrees of hyposmia or even anosmia. As a result, many are unaware of the odors that emulate from their bodies or clothing. Moreover, their spouses and caregivers may also suffer from reduced ability to sense foul odors, leading to persistent difficulties. As more elderly individuals actively wish to engage in community activities, the impact of the social disability increases. Odors due to poor personal hygiene or urinary or fecal incontinence are well recognized, and even more problematic from a social standpoint than halitosis. Halitosis accompanies a number of disorders of the head and neck in individuals of all ages. Not all oral odor originates within the oral cavity. Some of these are the odors associated with nasal foreign bodies, ulcerated, necrotic cancers of the oral cavity or pharynx, foods such as garlic, or exhaled odor from systemic disease such as ammonia or ketones. Some medications can result in breath odor (1). The identification and management of these entities is not different in the elderly from that in the younger population. It must be remembered that the differential diagnosis of halitosis extends beyond the tonsillar pillars. The oral odor of halitosis most frequently arises from anaerobic and microaerophillic gram-negative organisms that release volatile sulfur compounds (VSC), chiefly methyl mercaptan and hydrogen sulfide. These organisms include Treponema denticola, Porphyromonas gingivalis, Prevotella intermedia, Bacteroides forsythus, and Fusobacteria (2). These organisms reside deep in periodontal crevices, in the debris 261

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trapped by the papillae on the dorsum of the tongue, and in tonsillar crypts. A study of healthy subjects suggests that a quarter of healthy individuals are colonized with organisms that release volatile sulfur compounds. Occasionally patients will present with severe periodontal disease such as necrotizing ulcerative gingivitis (Vincent’s disease), but more commonly there is no specific oral lesion identified (3). Tongue coating varies among individuals as well as varies from time to time within the same individual. The thickness of the coating correlates with the degree of colonization of VSC-releasing organisms. Any lesion in the oral cavity that results in bleeding will lead to halitosis, including tumors, gingivitis, or dental care such as extractions or periodontal surgery. A study of institutionalized elderly in Japan demonstrated that there were higher levels of malodor as well as increased numbers of patients with oral colonization of Candida sp. and Staphylococci when compared with age-matched community dwellers (4). The authors correlate these changes with the poor oral care often provided to institutionalized elderly patients as well as the likelihood that many of the institutionalized are unable to perform their own oral care. They also note that Candida sp. are particularly adept at adhering to denture material; hence, Candida overgrowth on dentures often occurs in an institutional environment without adequate denture management policies. These investigators also measured sulfur compounds in saliva and noted that the levels were twice as high in institutionalized than community-dwelling elderly (4). Although both Candida overgrowth and VSCrelated oral malodor were found in higher numbers in the institutionalized, it was not clear whether any cause and effect relationship existed.

APPROACH TO THE PATIENT WITH HALITOSIS As with any complaint, the investigation of halitosis begins with a history and physical examination. Duration, timing, and character of the halitosis should be determined. The history of dental care and dental diseases should be obtained, particularly any recent dental work. A history of oral bleeding, as well as any symptoms that suggest the presence of specific oral or pharyngeal lesions should be elicited. The patient should be questioned regarding xerostomia as well as medications, particularly those that may result in xerostomia. The physical examination will include not only inspection of the oral cavity, but nasal endoscopy and examination of the hypopharynx as well. A search for adherent oral debris or other evidence of poor swallowing function should be done. The condition of the remaining teeth and presence of deep periodontal pockets or root caries should be noted. If the patient wears dentures they should be removed and examined for adherent debris. Throughout the exam, the physician should be alert to tell-tale odors that may indicate other diseases, such as necrotic tumor or ammonia due to liver failure. Laboratory testing is generally not required. Cultures are of little use, except perhaps in immunocompromised patients or others with unusual circumstances.

TREATMENT OF HALITOSIS Halitosis is best managed by mechanical means. In nearly all patients, this means standard oral care to include brushing, flossing, and mouth rinses. Elderly patients

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who are being fed by tube represent a particular problem as the lack of oral feeding results in reduced mechanical cleansing of the oral cavity by mastication and rinsing. Moreover, in many instances, these individuals suffer from other disabilities that make it unlikely that they would be able to administer their own oral care. Systemic antibiotics are not indicated for the management of halitosis. It is important to recognize that the oral cavity cannot be sterilized, and that systemic antibiotic therapy will only result in a temporary alteration of the flora. Topical antibacterial agents are, however, effective, and are used widely for a variety of oral indications. The most commonly used by dentists is chlorhexidine (Periodex1) which is highly effective, reducing VSC levels by more than 50% when combined with brushing (5). Unfortunately, over half of patients using chlorhexidine report a change in the taste of food and a quarter report burning, and it has been reported to cause staining. Hence, this product is better reserved for short-term use following dental work than for chronic use. Most individuals with halitosis chose to use an over-the-counter (OTC) mouth rinse, of which dozens exist. Most of these do reduce levels of VSC temporarily, although the duration varies. A blinded comparison of four commercially available OTC products with 0.2% chlorhexidine in normal subjects revealed that all resulted in lower levels of VSCs than did a water–alcohol control group; however, none were as low as the chlorhexidine group (6). There was minimal variability among the commercially available products. Based on the side effects of chlorhexidine, the authors recommend that patients use of any of the OTC commercial products instead. Mechanical cleaning of teeth and interdental crevices with brushing and flossing is the most effective treatment strategy. If there are marked levels of calculus and plaque, dental referral for cleaning and scaling is necessary. Mechanical cleansing of the tongue has been demonstrated to be effective in reducing oral malodor (5). Tongue scrapers (Fig. 1) are commercially available and should be recommended to patients with extensive tongue coating. The scraper should be placed as far

Figure 1 Photograph of a commercially available tongue scraper. The device is placed as far posteriorly as tolerated and drawn forward to scrape the debris off the papillae of the dorsum of the tongue.

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posteriorly as feasible, and then lightly drawn forward. The process is repeated until no further debris is brought out. Their use is uncomfortable for some due to gagging, but with practice most patients can find a location which is tolerable.

CONCLUSION Halitosis is a common problem affecting all individuals at some time. Elderly individuals are at greater risk due to xerostomia, dental disease, and reduced ability to perform oral care. The resultant social disability can be severe and impair functioning within society; hence, evaluation and management are warranted. The odor of halitosis is due to volatile sulfur compounds that are fermentation products produced by a number of anaerobic and microaerophillic gram-negative organisms. These organisms reside in dental crevices, tonsillar crypts, and in the coating on the dorsum of the tongue. Evaluation of affected individuals should include a complete head and neck examination to rule out other causes of the malodor. Mechanical cleansing of oral cavity surfaces is the mainstay of treatment, and is temporarily aided by limited use of OTC mouthwashes. Dental evaluation is required for some patients, particularly those with necrotizing ulcerative gingivitis. Daily oral care by caregivers is necessary for patients with inability to perform these activities of daily living on their own.

REFERENCES 1. Murata T, Fujiama Y, Yamaga T, Miyazaki H. Breath malodor in an asthmatic patient caused by side-effects of medication: a case report and review of the literature. Oral Dis 2003; 9:273–276. 2. Scully C, El-Maaytah M, Porter SR, Greenman J. Breath odor: etiopathogenesis, assessment and management. Eur J Oral Sci 1997; 105:287–293. 3. Coventry J, Griffiths G, Scully C, Tonetti M. ABC of oral health: periodontal disease. BMJ 2000; 321:36–39. 4. Honda E. Oral microbial flora and oral malodour of the institutionalized elderly in Japan. Gerodontology 2001; 18(2):65–72. 5. Quorynen M, Zhao H, van Steenberghe D. Review of the treatment strategies for oral malodour. Clin Oral Invest 2002; 6:1–10. 6. Garvalho MD, Tabchoury CM, Cury JA, Toledo S, Nogueira-Filho GR. Impact of mouth rinses on morning bad breath in health subjects. J Clin Periodontol 2004; 31:85–90.

24 Oral Health Care of the Elderly Patient Azfar A. Siddiqui School of Dental Medicine, Department of Maxillofacial Prosthetics, University of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION The quality of life for the geriatric adult is often impaired by poor oral health. Oral diseases and disorders affect health and well-being throughout life; however, they have a disproportionate effect in vulnerable populations such as the aged. The elderly geriatric population in the United States is the most rapidly growing population segment in the country. Currently, over 12% (32 million) of the U.S. population is aged 65þ years and this population will increase to 23% by the year 2040 (68 million). Nearly two million of those over the age of 65 are dependent for at least part of their care and reside in nursing homes. Many of these elderly will have remaining teeth, and the rest will have dentures. Dental conditions will affect their quality of life, nutritional status, and the management of chronic illnesses.

DENTAL LOSS IN THE ELDERLY In developed countries, elderly patients no longer accept the removal of natural teeth and provision of dentures as a component of normal aging. Over the past century, there has been a steadily increasing number of elderly people with retention of heavily restored natural teeth, even into old age due to improved dental care. A recent report from the Division of Oral Health at the Centers for Disease Control (CDC) notes that more than one-half of elderly adults have retained most of their teeth (missing five or less), with a range of 27% in West Virginia to 64% in Utah (1). Elderly patients today are much better informed of the various treatment options available to retain natural teeth and have the time, resources, and willingness to devote to their maintenance.

CHANGES IN ORAL DENTAL HEALTH WITH AGING Common pathologic conditions of the oral cavity occur with aging due to a combination of systemic, local, and lifestyle factors. Apart from the aging process itself, these factors include diseases of the teeth, bony supporting structures, gingival tissue, and 265

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salivary glands, all of which lead to dental loss. Caries in remaining teeth may result from poor nutrition, poor manual dexterity and inability of keep teeth clean, and failure of routine dental treatment. Xerostomia contributes to caries, and may be due to systemic disease or prior radiotherapy for head and neck cancer. Disease of the musculoskeletal structures, such as periodontal disease or apical cysts and abscesses, may promote alveolar bone resorption. Systemic conditions such as diabetes, stroke, HIV/ AIDS, and Alzheimer’s can predispose patients to develop oral disease. Social factors that contribute to dental disease and tooth loss include history of smoking, alcohol, and drug abuse. Poor oral health, often related to hygiene, leads to caries and periodontal disease. Infective organisms colonizing the oral disease can gain entrance to the bloodstream, seeding distant vulnerable sites such as damaged heart valves. Oral secretions with high concentrations of pathogenic bacteria may be aspirated into the lungs and result in life-threatening lung abscesses or pneumonia. Recent data suggest that some chronic degenerative diseases, such as endovascular disease, may be causatively related to a chronic inflammatory response to ongoing periodontal disease. Regardless of causation, oral disease is often correlated with a wide variety of systemic illnesses, lifestyles, and substances. Treatment needs of dentate patients are considerably greater than those who are edentulous, where maintenance can be relatively straightforward. The medical team caring for the patient must be able to evaluate, diagnose, and refer the patient for management of their dental needs if necessary. In the care-dependent population of elderly, caregivers must be instructed in oral care. Providing this care becomes both more critical as well as more difficult as aging progresses, due to increased xerostomia, increased retention of oral food particles following meals, and increased fragility of oral tissues. Otolaryngologists who are asked to evaluate the geriatric patient for other conditions should also routinely evaluate the status of oral care, and recommend intervention if necessary.

DENTURES IN THE ELDERLY Any oral examination must include removal of the dentures as well as an examination of the prosthesis that the patient may be wearing. These can range from fixed partial dentures or bridges to removable dentures. With poor oral hygiene or inability to keep the oral environment clean, this prosthesis can become a focus of bacterial and fungal colonization and can thus lead to undesirable oral conditions. Fixed partial dentures that have been placed for many years may predispose to recurrent caries, which if left unchecked can result in the loss of the prosthesis. Similarly, removable partial dentures and obturators need to be evaluated for proper fit so as not to cause chronic tissue trauma. Clips that attach to remaining teeth may result in erosion, leading to tooth loss, loss of fixation of the denture, and secondary tissue trauma secondary to chronic movement. Appropriate refitting may be necessary. If new fixation points cannot be identified, often full-mouth extraction and denture fitting may be the only viable option.

TREATMENT OF DENTURE PROBLEMS IN THE ELDERLY Mandibular height decreases as aging progresses in the edentulous patient due to loss of alveolar bone. This loss results in reduction of sulci adjacent to the mandible.

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Dentures that fit well following healing after extractions will need to be refit (relined) on multiple occasions during the life of the patient. Wear of dentures that do not fit results in excessive motion and pressure-induced ulceration from the denture flange. There is often significant adjacent reactive hyperplastic mucosa resembling neoplastic disease. These typically occur adjacent to the mandible in the depths of the buccalgingival and labial-gingival sulci. Pain is often a prominent complaint, and may impair eating to such a degree that weight loss ensues. Examination may initially suggest malignancy; however, if the patient leaves the dentures out for several weeks these lesions will resolve. Failure to resolve mandates biopsy. Comparison of the concave fitting surface of the denture with the shape of the remaining mandible will usually demonstrate the source of the pathology. Most edentulous patients require relining of their dentures every three to five years. Attempts to extend this interval lead to the development of secondary complications due to poor fit. Bone grafting to restore alveolar height is usually performed only on younger (fifth and sixth decade) edentulous patients due to donor-site morbidity. However, healthy elderly patients with severe bone loss and secondary denture dysfunction may occasionally be considered for grafting. Each patient should be considered individually, taking quality of life issues, comorbidities, and desires of the patient into account.

DENTAL CARIES Caries can develop at any age, but may be more problematic in the elderly. Loss of enamel is a result of etching caused by toxins secreted by gram-positive bacteria and is the first step in the process of decay. Caries can develop on either the occlusal surface or the sides of the teeth, as well as on roots exposed by loss of gingival and alveolar bone (gingival recession). If left untreated it will expose the dental pulp, resulting in dental nerve exposure with resultant pain, which can be severe. The open pulp cavity provides access for pathogenic organisms with the development of periapical abscess, spread of infection in the facial planes, and even cellulitis such as Ludwig’s angina. Root surface caries are more common than occlusal caries in the elderly population and occur due to exposure of the tooth roots, which are not protected by enamel. The elderly have many predisposing factors such as poor filling margins placed close to the gums, loss of alveolar bone, poor oral hygiene, and poor manual dexterity that contribute to rapid progression of root caries. One of the most important factors that can lead to the rapid development of caries and tooth decay is xerostomia. This can be a result of poor salivary function due to systemic conditions such as Sjogren’s disease or Alzheimer’s disease, or salivary dysfunction due to radiation therapy required for treatment of head and neck cancer. More than 400 drugs have been reported to decrease salivary flow, some of which may not be intuitive to the treating physician. In addition to diuretics, drugs such as tricyclic antidepressants, sedatives, tranquilizers, and antihistamines may contribute. A large number of elderly patients are on a variety of combinations of these and other medications that contribute to xerostomia; hence, they are more susceptible to rapidly developing tooth caries. Prevention of tooth loss becomes most important in patients who have received head and neck radiation. This is due to the fact that any extraction in the immediate post-radiation period can potentially result in osteoradionecrosis (ORN). Thus, a thorough and complete evaluation of the patient’s oral health prior to radiation

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therapy cannot be overemphasized. Management of the teeth in patients with cancer is discussed in a later section of this chapter.

TREATMENT Treatment of caries includes removal of decayed tooth structures, and replacement with restorative filling materials. Fluoride releasing filling materials are often employed when restoring root surface caries. Preventive measures should include regular follow-up evaluation and patient education, as well as educating caregivers or family members providing care to the elderly person. Fluoride mouth rinses, as well as custom fabricated fluoride delivery trays, are most useful for patients at high risk, such as those who have received radiation therapy to the head and neck region or have xerostomia of other causes. A thin bead sodium fluoride gel is placed in the tray, which is worn over the teeth for a minimum of five minutes daily before bedtime. The patient should avoid eating and rinsing for 30 minutes following treatment.

PERIODONTAL DISEASE Periodontal disease is a disease of the supporting structures of the teeth, namely the gingival tissues, alveolar bone, cementum, and the periodontal ligament. Periodontal disease can broadly be classified as either gingivitis or periodontitis. Gingivitis involves only the gingiva, whereas periodontitis involves the alveolar bone, periodontal ligament, and cementum. The major causative organisms are anaerobic gram-positive and -negative bacteria found in dental plaque. The quantity of organisms in this plaque is quite high, approaching the number that would be found in a pure culture of bacteria. Once entry to the soft tissues is obtained, local tissue sepsis ensues. Although age in itself is not a factor, multiple comorbidities common in the elderly can contribute to periodontal disease. These include systemic disease, medications, and patient’s ability to maintain good oral hygiene. Patients with diabetes demonstrate a higher prevalence of periodontal disease due to their propensity for slower healing. The prevalence of periodontal disease in the elderly population is possibly since more elderly patients are retaining their original dentition and are on more medications. Several medications frequently prescribed to the elderly may cause gingival enlargement including calcium channel blockers and the well-known phenytoin. Periodontal disease may affect both the oral and systemic well-being of the patient. Consequences of untreated periodontal disease include loosening of teeth with eventual tooth loss, pain, local sepsis, and systemic disease due to sepsis or reduced nutrition. Patients may be entrapped in the vicious cycle of periodontal disease leading to pain, reduced oral intake, nutritional deficiencies, immunosuppression, and poor healing, resulting secondarily in increased periodontal disease.

TREATMENT OF PERIODONTAL DISEASE Treatment of active periodontal disease includes topical and systemic antimicrobial therapy, mechanical removal of plaque by scaling/root planning, and surgical elimination of periodontal pockets if possible. However, patients who are immunocompromised, are frail and elderly, or have systemic diseases may be poor candidates

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for such procedures. In these patients, treatment should be limited to conservative therapy of superficial prophylaxis/root planning, and antimicrobial therapy. Extraction may often be the most conservative therapeutic option. Antimicrobial therapy for severe periodontal disease includes twice-daily mouth rinses with chlorhexidine gluconate 0.12% (PeriodexTM) and systemic therapy with an antibiotic with coverage of oral anaerobes such as metronidazole or clindamycin.

ORAL HEALTH OF CANCER PATIENTS A particularly vulnerable population is those elderly patients who have been diagnosed with head and neck cancer and are scheduled for either surgical or nonoperative therapy. These patients require a thorough oral evaluation prior to starting any treatment to ensure that needed prophylactic oral treatment is provided in order to minimize post-treatment complications. Patients with cancer of other sites who require systemic chemotherapy frequently develop severe oral sequellae, particularly mucositis. The intense mucositis and painful ulcerations of chemotherapy are usually relatively short-term and resolve over several weeks as the antiproliferative and immunosuppressive effects of the chemotherapy abate. However, over the short term the pain of the mucositis will impair the ability of the patient to maintain proper oral hygiene measures, resulting in worsening of any underlying periodontal disease. Oral care of these patients is difficult, but vital to their recovery. Immunosuppression and loss of the mucosal barrier predispose the patient to oral fungal infections, recurrent herpetic lesions, oral infections, gingival bleeding, or inflammation. Gingival conditions encountered in normal healthy individuals may require surgical intervention; however, such treatment may not be possible in an immunocompromised patient. Timing of needed dental treatment must be coordinated with other therapy, and is usually limited to that which is most necessary. Treatment-related complications can often be avoided or their severity reduced with a thorough dental evaluation and treatment prior to the initiation of either local radiation and/or systemic chemotherapy. The dentist evaluating a patient prior to therapy should be informed of the diagnosis as well as the planned treatment. Nonrestorable teeth and those with periodontal disease should be extracted. Dento-alveolar infections and oral mucosal conditions that may be asymptomatic at this stage should be addressed and effective treatment rendered prior to starting any cancer treatment. Failure to do so can result in complication arising during or in the immediate post-treatment period, when the patient is immunocompromised, and can be life-threatening. In most instances, the patient should be closely followed during and following therapy by a dentist familiar with the oral manifestations of cancer treatment. Patients scheduled to receive radiation therapy to the head and neck should be evaluated by a dentist prior to the initiation of treatment. The usual dose of head and neck radiation exceeds 6000 cGy, sufficient to result in long-term complications such as poor healing, osteoradionecrosis, and xerostomia. Any teeth with even minimal probability of causing problems within the next two years should be restored or extracted prior to radiation therapy. Extractions performed following radiotherapy have a potential to precipitate osteoradionecrosis and result in severe morbidity. Teeth with large carious lesions, periapical disease, and periodontal infections should be extracted.

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A decision regarding preservation of some teeth should be made based on the patient’s motivation and life expectancy, as well as the potential need for a future prosthesis for postoperative rehabilitation. If it is felt that the patient is highly motivated to keep his teeth clean, then required restorative treatment should be completed. Custom fluoride trays should be provided to the patient to reduce the incidence of radiation-induced caries. It is recommended that the fluoride trays should be worn after normal brushing for a period no less than five minutes every day, using sodium fluoride gel applied to the fitting surface of the trays. Extractions in the immediate post-radiation period should be avoided due to the possibility of developing ORN. The risk of ORN is greater in the mandible than in the maxilla and depends on the total dose of radiation, fraction size, and energy levels, as well as the direction of the beam and shielding used. Newer techniques (such as intensity-modulated radiotherapy) may reduce the dose to the bone and reduce the risk. If extractions or preprosthetic dento-alveolar surgery is performed prior to radiation therapy, a minimum delay of two to three weeks should be allowed prior to starting radiation therapy. Extractions that become necessary following completion of therapy present a significant problem. If possible, these should be performed within several months following completion of therapy during the hyperemic phase. Following this phase, however, radiation-induced microangiopathy results in reduced vascularity, relative tissue hypoxia, and reduced capacity for healing, particularly bone. Treatment with hyperbaric oxygen therapy has been demonstrated to be useful by increasing tissue oxygenation, promoting bone healing, and reducing complications in patients who require late extractions. Standardized protocols typically require 20 dives prior to surgery and 10 dives post surgery. Cost is often a significant issue for these patients. Perioperative antibiotics have been demonstrated to reduce the risk of ORN and are strongly recommended. Treatment of chemotherapy-induced mucositis is symptomatic and consists of analgesics, local irrigations, and mouth rinses. One common ‘‘magic swizzle’’ rinse is an emulsion made up of equal parts of BenadrylTM solution, KaopectateTM, and viscous lidocaine. Patients are instructed to keep the emulsion in the mouth for one minute, expectorate, and not to rinse. Treatment of oral fungal infections in immunocompromised patients should first aim at improving oral hygiene. If the patient is wearing removable dentures or obturators, these should be removed and worn only for meals. Often improvement in oral hygiene can result in resolution of the problem. If fungal infections still persist, the patient can be treated with a variety of medications including topical NystatinTM or ClotrimazoleTM, in suspension or troches, or systemic antifungal agents such as fluconazole.

DENTURE CARE Wearing a removable prosthesis is often uncomfortable for the patient during radiation/chemotherapy as well as in the postoperative period following surgery. However, in order to maintain swallowing and nutrition, their minimal use may be necessary. During this period, the patient may complain of loose or ill-fitting dentures. To help solve this problem while the patient is recovering, the dentures can be relined with a temporary soft liner. This is usually sufficient to improve function. Dentures may need to be relined several times to help the patient adjust

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to their prosthesis. If anatomic relationships have been altered by treatment, a new prosthesis may need to be fabricated after the patient has recovered. Denture wear is problematic for post-radiation patients due to lack of the lubricating effect of saliva. Denture wear can also lead to traumatic abrasions and ulcerations. Artificial salivary substitutes as well as constant sipping of water can improve the condition to some extent. Specialized prostheses such as obturators and mandibular repositioning appliances require care similar to that of conventional dentures. It should be noted that all dentures, no matter how well they are fabricated, accumulate plaque and provide an area for bacterial and fungal growth. Maintenance of oral hygiene becomes of paramount importance. Dentures and obturators should not be worn at night and should be cleaned and allowed to soak in either water or antiseptic mouthwash. This allows the tissues to recover as well as assists in mechanically removing bacteria from the denture surface. However, patients recovering from treatment are often unable to comply with oral hygiene measures. Under these circumstances, patients can develop fungal colonization of the oral appliances. Application of a few drops of nystatin oral suspension or a thin film of nystatin oral ointment to the fitting surface of the dentures after each meal often helps to reduce this colonization.

SUMMARY Oral health significantly impacts the quality of life as well as general well-being of geriatric patients. Elderly patients are particularly vulnerable to the development of oral and dental diseases, and may be adversely affected in a variety of ways. More elderly patients now retain their teeth into old age, and avoidance of dental and periodontal disease requires on-going maintenance, either by the patient, or, as they age, their caregivers. Those without teeth and dentures require periodic relining of their dentures to accommodate the changes in oral anatomy that accompany aging. Finally, those elderly patients who require treatment for cancer have even greater needs for oral care and prophylaxis to prevent serious complications.

REFERENCE 1. Gooch BF, Eke PI, Malvitz DM. Public health and aging: retention of natural teeth among older adults—United States, 2002. MMWR 2003; 52:1226–1229.

25 Burning Mouth David E. Eibling Department of Otolaryngology—Head and Neck Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

INTRODUCTION Burning mouth syndrome (BMS) is a chronic pain condition of unknown etiology involving the mouth, usually the tongue, with no obvious source. In classic BMS, no cause for the symptom can be identified by history, physical examination, or laboratory testing. A neuropathic pain process involving the distribution of the fifth and ninth cranial nerves is the most likely etiology.

REPRESENTATIVE CASE A 72-year-old lady presents with complaints of a ‘‘burning mouth.’’ This pain has been present for more than a year, and has not changed appreciably since it was first noted. The lady has seen the primary care physician as well as the dentist and no known cause has been identified. Over-the-counter medications including topical anesthetics were unsuccessful in relieving the pain, and in fact seemed at times to make the pain worse. The patient cannot remember exactly when the pain began, although does believe that a viral upper respiratory infection might have occurred at the time of onset. The pain is more or less constant but seems to be worse at the time of awakening at night. The patient has no symptoms of sore throat, dysphagia, reflux, swelling, ulcerations, or bleeding, and relates no past history of herpes infections nor apthous ulcers. The past medical history of the patient is unremarkable and no medications other than Fosamax were used by the patient. Specifically there is no history of arthritis, hypertension, cancer, diabetes, or cardiac disease. The patient does not associate the onset of pain to the medication, which has been taken for a number of years. The patient is a lifetime nonsmoker and nondrinker. The lady is widowed, her husband having died approximately five years ago. All children of the patient live out of state, but the patient keeps in contact with them with weekly telephone calls. The patient is active in church activities and is also a member of several volunteer organizations in the community. The patient is not believed to be depressed and describes the pain as more of a ‘‘giant frustration’’ that adversely affects her quality of life but is not debilitating. 273

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EPIDEMIOLOGY OF BMS BMS is a frustrating (and often debilitating) condition to both the patient and physician. By the time the average patient arrives at the otolaryngologist office, they have usually tried a number of over-the-counter medications, been treated by their primary physician with a variety of treatments, and may have seen their dentist. In typical cases, the physical examination is completely normal and no underlying cause can be identified. Specifically, there is no past history of herpetic ulcerations, prior oral cavity lesions, surgery, or radiation therapy. Salivary flow is normal, with moist mucosal surfaces, although the patients may complain